Cystic lymphangioma is a very rare condition generally accepted to be the result of a developmental malformation of the lymphatic system. Usually seen in children, it occurs infrequently in adults. The neck (75%) and axillary regions (20%) are the most common locations of lymphangioma, but it can occur in the retroperitoneum, mediastinum, mesentery, omentum, colon, pelvis, groin, bone, skin, scrotum, and spleen. We present a cystic lymphangioma of the spleen with emphasis on its rarity in this site and problems of differential diagnosis with hydatid disease.