We report the rare case of an intraductal papillary mucinous tumor (IPMT) in a man younger than 30 years of age. The patient was admitted with upper abdominal pain and an elevated amylase level of 662 IU/l. Ultrasonography showed a cystic mass in the pancreatic body and endoscopic retrograde cholangiopancreatography (ERCP) revealed a dilated pancreatic duct with a filling defect communicating with the tumor. He was successfully treated by segmental resection, which seems to be the best surgical option for pancreatic body tumors since it results in long-term survival and preserves as much pancreatic parenchyma as possible. Nevertheless, it can only be done in the absence of additional nodules along the pancreatic duct. A pathological diagnosis of intraductal papillary adenocarcinoma of the noninvasive type was confirmed, and both stumps were free of tumor.