ABSTRACT
Pancreatic divisum is the most common anomaly of the pancreas. Pancreatic divisum associated with malignant tumor is rarely seen. The ampulla of Vater is formed by the union of the common bile duct and the pancreatic duct. The incidence of malignant tumor in the ampulla of Vater is very low. Adenomas and carcinomas are more common. Neuroendocrine tumors originate from enterochromaffin cells of the neuroendocrine system. Ampullary neuroendocrine tumors are very rare and preoperative diagnosis is very difficult. In the literature, the emphasis on imaging characteristics of similar cases is limited. Herein, we present a rare case of pancreatic divisum associated with ampullary neuroendocrine tumor and its characteristics of computed tomography and magnetic resonance imaging.