Cystic teratomas of the pancreas constitute an extremely rare entity with only nine cases, to our knowledge, described in the world literature. Symptoms are usually due to the compressive effects of the tumor on the neighboring organs. They should be considered in the differential diagnosis of slow-growing benign pancreatic cysts. We describe a 25-year-old woman with a pancreatic teratoma who was operated on in 1976 with the diagnosis of calcified pancreatic cyst. The diagnostic and surgical procedures are described, as well as a 14-year follow-up. The previously published cases are reviewed and the differential diagnosis is discussed. Early diagnosis and the need for total tumor resection are emphasized.