Mucinous cystic neoplasms (MCN) of the pancreas occur mostly in females and are potentially sex hormone-sensitive. MCNs are more common in women with a peak incidence in the fifth decade. They are usually located in the distal pancreas, and frequently grow up to a size of 8–10 cm. We report a case of giant MCN of the pancreas in a 31-year old female patient, occupying entire left abdomen, successfully treated with distal pancreatosplenectomy. Histology demonstrated MCN with low-grade dysplasia. At 4 year of follow-up, the patient is free of disease.