A 9-year-old Chinese boy with abdominal pain and an abdominal mass was found at laparotomy to have carcinoma of the pancreas. Histologically, this was an acinar cell carcinoma, which is an extremely rare condition, especially in childhood. The patient survived for ten months after pancreatoduodenectomy and choledochojejunostomy. A review of the literature showed that in contrast to adults, jaundice was an uncommon finding in children with pancreatic carcinoma, and that an enlarging abdomen or an abdominal mass was the initial feature in the majority of patients. Prognosis was poor even with treatment, the longest survivor having lived for five years after pancreatoduodenectomy.