Primary neoplasms of the pancreas are rare in children. One variant of these tumors is pancreatoblastoma, comprising 0.5% of epithelial tumors of the pancreas. It usually affects children at 1-8 years of age, with quite equal sex ratio. The tumor can be found at any site of pancreas, but it most commonly arises in the head of pancreas. Histopathologically, pancreatoblastoma is an encapsulated tumor with distinct organoid structures and sometimes squamoid corpuscles. Acinar cells with zymogen granules are occasionally found. The tumor has favorable prognosis. We present a 14-year-old female who was admitted due to prolonged jaundice for about 2 months. Physical examination revealed pale conjunctiva, yellowish skin color and hepatosplenomegaly. Abdominal sonography and CT scan showed dilated common bile duct and a tumor mass about 2 x 2 cm in dimension located a pancreatic head. She received surgical operation to relieve obstructive jaundice and later Whipple's operation for radical resection. Six months after operation, the patient received the examination of abdominal sonography, CT scan and gallium tumor scan but there was no evidence of local recurrence or distant metastasis. Now the patient is living well for more than one year. Because of its rare occurrence, we demonstrate this case and review the literature.