A 33-year-old woman who presented with epigastric discomfort and diarrhea underwent an abdominal ultrasound (US). This investigation and subsequent contrastenhanced computed tomography, magnetic resonance imaging and endoscopic US with fine needle aspiration (FNA) revealed a 40 mm well-circumscribed mass in the uncinate process of the pancreas. Findings were suggestive of a mucinous or solid-cystic pseudopapillary tumor of the pancreas, although other lesions such as a nonfunctioning neuroendocrine tumor could not be ruled out. FNA samples were negative for malignant cells, but of limited value due to poor cellularity. It was decided to surgically remove the tumor because malignancy could not be discounted. Multiple intraoperative biopsies were suggestive of mesenchymal tumor and consequently a conservative resection (uncinatectomy) was performed. The postoperative course was uneventful. The definitive diagnosis was ganglioneuroma. Immunocytochemistry showed positive staining with vimentin, S-100 protein, neurofilament and neuron-specific enolase. Ganglioneuroma is a rare benign tumor that can also present as a pancreatic tumor. Uncinatectomy is feasible, safe and a good surgical technique for the treatment of nonmalignant tumors located in the uncinate process of the pancreas.