家族性颌骨纤维异常增殖症也称巨颌症,是一种良性自限性病变,较为少见,目前认为是常染色体显性遗传性疾病。病变发生于幼儿时期,男性多见。本病主要侵犯下颌骨,同时波及上颌骨并呈特殊的多囊性病变者更为少见,特报告如下。患儿男,5岁。双侧上,下颌骨均呈无病性膨胀三年,以右侧为甚。追问家族史,其父亦有类似病变。临床表现:双侧颞部、眶下区、下颌角及下颌体区对称性肿大,两眼轻度上翻,露 Familial maxillary fibrous dysplasia, also known as giant jaw syndrome, is a benign, self-limited lesion that is less common and is currently considered an autosomal dominant inherited disorder. Lesions occur in early childhood, more common in men. The main violations of the disease mandibular, while affecting the maxilla and polycystic lesions were more unusual, especially reported as follows. Children male, 5 years old. Both sides of the mandible were non-pathological swelling for three years, to the right is even worse. Asked family history, his father also have similar lesions. Clinical manifestations: bilateral temporal, infraorbital area, mandibular angle and mandibular body area symmetry swelling, slightly upturned eyes, dew