A case of exocrine pancreatic carcinoma in a 14-year-old boy is reported. The primary site of the tumor was in the head of the pancreas, and pathologic features were consistent with an anaplastic lesion. Ten years after curative resection the patient is still living. Extensive review of the literature has revealed 27 other cases of pancreatic carcinoma in children under 15 years of age. Aggressive surgical treatment is emphasized by the long survival observed in four patients who underwent radical operations and by this case report.