A rare case of Frantz's tumor with liver metastasis is reported. An 11-year-old girl had local resection of a Frantz's tumor in the head of the pancreas. Ten years later, she had a recurrence in the pancreatic head, and two liver metastases were also disclosed at reoperation. Pancreaticoduodenectomy and enucleation of metastatic lesions in the liver were performed. Six years after the second operation, she is well and free of disease. A review of 174 cases of Frantz's tumor disclosed that metastatic disease in the pediatric age group is extremely rare and yet the prognosis is favorable. On the other hand, the outcome in older patients, especially those over 40 years old, is very poor.