Abstract: Retroperitoneal ganglioneuroma is a rare neuro genic benign tumor. The prognosis of patients was good when the tumor was completely resected, while the surgical procedure is complicated. In March of 2013, a male patient with complex retroperitoneal ganglioneuroma was treated at the First Affiliated Hospital of Chongqing Medical University. A hypoechoic solid lesion (size, 6.5 cm×4.5 cm) adjacent to the head of the pancreas was detected by color Doppler ultra sonography 9 months ago, and no any other clinical symptoms were detected. Perioperative abdominal computed tomography and the surgery confirmed that the tumor (size, 8.5 cm×7.5 cm×4.5 cm) was located beneath the pancreas, encompassing the celiac artery, hepatic artery, splenic artery and superior mesenteric artery, surrounding the head and uncinate process of the pancreas, making it impossible to be separated. The tumor was closely connected with the portal vein, superior mesenteric vein, splenic vein and left renal vein. The tumor was separated from the major blood vessels, the body and tail of the pancreas, while the tumor could not be resected from the pancreatic head, and thus tumor resection and pancreaticoduodenectomy were performed. The surgery was extremely diffcult, but the complete removal of tumor was successfully achieved without excision of the major blood vessels and the patient recovered well.