An 18 year-old resident of Zagreb was admitted to our hospital with intermittent pain in the right subcostal region. On examination, a palpable resistance was found in the upper abdomen. After extensive clinical and laboratory tests, a tumor of the pancreatic head, 80-85 mm in diameter, was verified. Cytologically, a diagnosis of microcystic adenoma of the pancreas was established. The patient underwent a cephalic pancreatoduodenectomy with preservation of the pylorus. Six months later the patient was no longer on a diet and, at follow-up, 3 years after surgery, she is symptom-free and feeling well.