Hydatidosis is endemic in many Mediterranean countries, the Middle East, South America, Australia, New Zealand and Africa. It is caused by an infection from the Echinococcus granulosus larvae which can lead to the development of cysts. The most frequently affected organ is the liver. Splenic involvement alone is very rare. In this article, a case of an isolated giant splenic hydatid cyst is reported. The patient underwent splenectomy. The diagnosis of a hydatid cyst was confirmed by histopathology. This case suggests that hydatid disease should be considered as a differential diagnosis in every patient with a cystic mass of the spleen in endemic areas.