Epidermoid cysts of the spleen that occur mainly in persons less than 20 years of age are rare. A 14-year-old girl presented with left upper quadrant pain. Abdominal ultrasonography revaled a well-defined, 10 x 9 x 8 cm cyst arising from the splenic hilus. Splenectomy was performed and postoperative recovery of the patient was uneventful. Pathological examination of the specimen revealed epidermoid splenic cyst. Partial splenectomy for removal of uncomplicated splenic cysts is preferred to total splenectomy because of protected splenic function and successful therapy. However, total splenectomy is performed for cysts located at the splenic hilus or for large cysts that would not allow the preservation of splenic tissue.