Hydatid disease, a zoonosis, occurs worldwide but its prevalence is high in those countries where sheep and cattle raising constitute an important industry due to close association between man, sheep and dog. Most common sites of involvement by hydatid disease are liver followed by lung. Splenic involvement by hydatid disease is very rare accounting for only 0.9% to 8.0% of all cases. Other rare sites include heart, pancreas and muscles. We report a case of histopathologically confirmed primary hydatid cyst spleen in an 18 year old male, which is a rare entity. Hydatid disease should be considered in the differential diagnosis of cystic lesions of spleen.