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Echinococcosis, or hydatid disease, is a zoonotic parasitic infection caused by the larval stage of Echinococcus granulosus. Although the liver and lungs are the most commonly affected organs, primary isolated splenic hydatid cysts are rare, accounting for less than 2% of all cases even in endemic regions. Diagnosis can be challenging due to non-specific clinical presentations.We report a female in her late 20s from Rampur district in Uttar Pradesh who presented with dull, intermittent pain in the left upper abdomen for 3 years. Imaging revealed a well-defined cystic lesion within the splenic parenchyma without involvement of other organs. Serological testing for hydatid disease using Echinococcus IgG ELISA was positive. The patient underwent laparoscopic splenectomy and histopathological examination confirmed the diagnosis of a hydatid cyst. Postoperative complications, including bilateral pleural effusion, pancreatic fistula and surgical site infection, were managed conservatively. The patient recovered well and remained asymptomatic at the 12-month follow-up with no evidence of recurrence on imaging.Primary isolated splenic hydatid cyst is an uncommon manifestation of echinococcosis and should be considered in the differential diagnosis of cystic splenic lesions, particularly in patients from endemic regions. Imaging, serological testing and histopathological evaluation are key to establishing the diagnosis. Splenectomy remains the treatment of choice.