Cystic tumours of the spleen are generally rare, and a parasitic origin is relatively unlikely. The present case report shows, however that when a splenic cyst is found, the differential diagnosis must always consider the possibility of echinococcosis. We report the case of a patient suffering from a cystic lesion of the spleen where surgery and histopathology yielded the diagnosis of splenic echinococcosis. Abdominal pain in the left upper quadrant and splenomegaly detected by simple abdominal radiology are the most commonly found indicators for this disease. The treatment should be surgical, attempting to preserve as much splenic tissue as possible, although conservative treatment is frequently unfeasable due to massive involvement of the spleen. Although rare, splenic hydatidosis should be included in the differential diagnosis when a cystic splenic lesion is identified with sonography or CT scan.