Advances in foetal imaging have increased our detection rate of craniofacial abnormalities in utero. Nasal glioma is a rare, benign, congenital facial defect. Once detected, further imaging is required to assess for intracranial communication, the presence of additional defects, determine the patency of the aerodigestive tract and decide on timing of delivery. The authors review the current literature on diagnosis and management of nasal glioma in this rapidly advancing field of craniofacial anomalies detected in utero. Literature search of EMBASE and MEDLINE databases yielded 594 articles, which were screened by 2 independent reviewers. A total of 7 papers were selected after exclusion. There have been seven cases of prenatally diagnosed nasal glioma. The earliest of these was detected at 20 wk gestation. The majority were investigated with foetal magnetic resonance imaging(MRI) to establish any intracranial communication or bony defects. Ultrasound monitoring, doppler waveform and 3D rendered images were utilised to delineate the lesion, monitor growth and differentiate potential diagnosis. Postnatal MRI is favoured by most to re-evaluate the lesion and aid surgical planning. Surgical resection was performed within the first few months of life. Diagnostic uncertainty wasseen in all cases, until formal histology was obtained, emphasising the challenges, and need for early appropriate specialist input. Whilst the prenatal detection of craniofacial abnormalities increases, there remain diagnostic challenges in differentiating prenatal congenital midfacial defects in utero. These defects are best investigated and monitored using prenatal ultrasound and MRI, to narrow the differential diagnosis, guide timing of delivery and allow for appropriate surgical planning. Prenatally detected nasal glioma, may only be confirmed on histology and families must be counselled appropriately to prepare them for the possible alternative diagnoses. Early surgical resection was undertaken to achieve more favourable aesthetic outcomes, reduce complications of ocular development and provide definitive histological diagnosis. Advances in foetal imaging have increased our detection rate of craniofacial abnormalities in utero. Nasal glioma is a rare, benign, congenital facial defect. Once detected, further imaging is required to assess for intracranial communication, the presence of additional defects, determine the patency of the aerodigestive tract and decide on timing of delivery. The authors review the current literature on diagnosis and management of nasal glioma in this rapid advancing field of craniofacial anomalies detected in utero. Literature search of EMBASE and MEDLINE databases ofielded 594 articles, which were screened by 2 total reviewers. A total of 7 papers were selected after exclusion. There have been seven cases of prenatally diagnosed nasal glioma. The earliest of these was detected at 20 weeks. The majority were investigated with foetal magnetic resonance imaging (MRI) to establish any intracranial communication or bony defects. Ultrasound monitoring, doppler waveform and 3D rendered images were utilised to delineate the lesion, monitor growth and differentiate potential diagnosis. Postnatal MRI is favored by most to re-evaluate the lesion and aid surgical planning. Diagnostic uncertainty wasseen in all Cases, until formal for early input specialist. emphasize the challenges, and need for early appropriate specialist input. Whilst the prenatal detection of craniofacial abnormalities increases, there remain diagnostic challenges in differentiating pre -atal congenital midfacial defects in utero. These defects are best investigated and monitored using Prenatal ultrasound and MRI, to narrow the differential diagnosis, guide timing of delivery and allow for appropriate surgical planning. Prenatally detected nasal glioma, may only be be sure on histology and families must be counselled appropriately to prepare them for the possible alternative diagnosis. Early surgical resection was undertaken to achieve more favourable aesthetic outcomes, reduce complications of ocular development and provide definitive histological diagnosis.