Duodenum and pancreas hemolymphangioma is a type of rare benign neoplasm. Pancreatoduodenectomy (PD) is a procedure known to be associated with high risk of complication. A review of literature published before December 2015 found only five hemolymphangioma cases that were treated with PD. We presented a case of a hemolymphangioma in a 15 years old male patient with a tumor of 16x11x8 cm(3) in size localized at the duodenum and pancreatic head. The patient had intermittent epigastric pain for 2 weeks before he was admitted to our hospital. Physical examination showed a huge mass in the right upper abdomen. Computed tomography (CT) indicated that the lower end of common bile duct and the second part of duodenum were extrinsically surrounded by a large retroperitoneal tumor. PD was performed and tumor was removed completely. Post-surgical diagnosis confirmed the tumor originated from duodenum and pancreas. There were no major complications in perioperative period, and the patient was discharged at the 14th day post-surgery. The patient was in good physical condition during followup visit 6 months after surgery. In conclusion, although PD is a procedure with high risk of postoperative complications, for some cases with benign lesions, it can still be a choice for a complete excision, which can significantly decrease the opportunity of recurrence and increase the rate of cure.