Although splenic involvement alone in hydatid disease is very rare, spleen is the third most common organ involved in hydatid disease. The rarity of splenic hydatid disease poses a diagnostic challenge for clinicians, particularly in non-endemic areas. The hydatid cyst can present as a simple cyst without having the classic serological and imaging features, and can lead to life-threatening complications like anaphylaxis. Hydatid disease of spleen should be considered in differential in every patient in endemic areas with cystic lesion of spleen until proved otherwise.