Background: Cystic Echinococcosis (CE) is one of the most prevalent important neglected zoonotic diseases caused by the larval form of the tapeworm, Echinococcus granulosus, and it represents a significant public health threat in many Mediterranean countries like Egypt. Human acts as the accidental intermediate host where the larval stage, hydatid cyst is formed. While liver and lung constitute the most commonly involved sites for primary cysts, other organs are occasionally affected. Primary hydatid cysts of the spleen are rarely present.  Patients and methods: This study reported an uncommon case of multiple primary hydatid cysts of the spleen in a 55-year-old man presented with pain in the left hypochondrium. A history of close contact with dogs was reported. The initial diagnosis was based on the abdominal ultrasonography and confirmed by the computed tomography (CT) scan and the serological detection using indirect hemagglutination test (IHAT) in the clinic. Results: The ultrasonography and CT scan revealed subcapsular hydatid cyst in the spleen with peripheral small daughter cysts. Serologically, hydatid cyst positive patient serum was recorded at 1:1280 titer by IHAT. Total splenectomy was successfully done, and the patient was discharged and prescribed with a prophylactic albendazole. Conclusions: ultrasonography, computerized tomography and serology are useful diagnostic tools for hydatidosis and the disease is considered a public health challenge and needs accurate differential diagnosis from any cystic mass/s in the abdomen.