BACKGROUND Extrahepatic and extrapulmonary localizations of cystic echinococcosis (CE) are rare and the incidence of splenic involvement is seen in 1.0% to 3.3% of all cases in the endemic areas of the Middle East and Asia. The diagnostic pathway consists of a detailed travel history, physical examination, abdominal ultrasonography, computed tomography, and serological tests. The efficacy of perioperative administration of albendazole (400 mg twice a day) has been proven; however, the appropriate surgical procedure for the treatment of giant, centrally located splenic hydatid cysts remains controversial. CASE REPORT We present the case of a 49-year-old woman referred to our hospital for a suspected isolated splenic hydatid cyst causing a compression of the right kidney, stomach, and the tail of the pancreas. She reported chronic pain in the left upper quadrant and a history of contact with animals. She underwent open splenectomy via a medial to lateral approach to minimize manipulation of the spleen. In addition, she received perioperative parasitostatic drug therapy with albendazole. The postoperative period was uneventful and the histologic analysis confirmed the diagnosis. CONCLUSIONS The spleen is a rare location for isolated CE, especially in non-endemic areas and must be considered in the differential diagnosis of splenic cystic masses. Surgical resection remains the most effective treatment that completely resolves this condition. A comparison of randomized trials is needed to compare the recurrence rates between splenectomy and spleen-preserving procedures in the treatment of giant splenic hydatid cysts.