The authors present a case of epidermoid splenic cyst in masculine patient of 13 years, that presented decrease of appetite, fever and alimentary vomits and tangible abdominal mass in the physical exam. Abdominal ultrasonography demonstrated expansible lesion in the topography of the spleen, and computerized tomography revealed intrinsic homogeneous lesion of the spleen, without communication with pancreas. To the laparotomy, cystic mass of great extension was observed, submitted total esplenectomy, being diagnosed in the histopatological exam epidermoid splenic cystic. In the follow-up patient developed with disappearance of the symptoms. The epidermoid splenic cyst are rare, they can be seen mainly in children and young adults, what proposes a congenital origin for this disease.