Pancreaticoduodenectomy is the treatment of choice for adult periampullary lesions. However there has been no studies on the clinical outcomes of pancreaticoduodenectomy in children. To evaluate the clinical outcomes, records of 13 patients who underwent pancreaticoduodenectomy, from 1989 to 2009, at Seoul National University Children's Hospital were reviewed. Mean follow up period was 83 (2-204) months, the male to female ratio was 1:3.3, and the mean age was 11 (2-14) years. Ten patients underwent PPPD and 3 patients had Whipple's operation. The postoperative diagnosis included solid pseudopapillary tumor (9), cavernous hemangioma (1), pseudocyst (1), benign cyst (1), pancreatic disruption (1). Two patients developed postoperative adhesive ileus and among them one patient required operative intervention. Four patients required pancreatin supplementation due to steatorrhea and other gastrointestinal symptoms. There were no postoperative mortality during the follow up period and no evidence of recurrence in SPT patients. This study demonstrates that the pancreaticoduodenectomy procedure in children is not only feasible but also safe, with no mortality and an acceptable complication rate.