Cystic disease of the spleen is not frequently encountered in surgical practice. It is broadly classified as parasitic and non-parasitic, the latter being a rare entity. In this case report, an epidermoid cyst of spleen led to acute abdomen in young age pregnant woman is presented.A 26-year-old woman with 15 weeks gestational age second pregnancy referred to emergency ward with left upper quadrant pain and tenderness that ultra sonography revealed large cyst (100х110mm) with internal echo in spleen in favor of hydatic cyst or abscess. The patient was managed medically for one week but developed her abdominal pain, epigastric fullness, anorexia and fever. New sonography was done for her and large cyst was detected in spleen (120х110mm). She had severe pain accompanied with anorexia and vomiting. Fetus was normal in sonography. Exploratory laprotomy with splenectomy was performed. Splenomegaly (200х130х90mm) with a cyst without any adhesion and inflammation was detected. Histopathology examination showed epidermoid cyst of spleen. (140х10х90mm). Though splenic epidermoid cyst is an uncommon entity, it should be considered in the differential diagnosis of an abdominal mass in a young individual. An attempt should be made to preserve the spleen provided there is adequate parenchyma otherwise splenectomy is the rule.