A 26-year-old male presented with a left upper abdominal mass of one year's duration. Ultrasonography revealed a cystic lesion arising from the lower pole of the spleen. Total splenectomy was done and pathological examination of the cyst confirmed a true cyst with mesothelial lining without squamous metaplasia. The epithelial linings of these true cysts ranged from flattened low cuboidal, low columnar to squamous type and unilayered or stratified. The pathogenetic hypotheses as well as clinicopathological features of this rare lesion, which is usually found in children and young adults, were reviewed.