Hydatid cyst of the kidney is one of the rare condition caused by the larval stage of Echinococcus granulosus. The incidence of renal involvement is about 2% of all hydatid disease in humans and isolated renal involvement is extremely rare. Hydatid disease is frequent in endemic regions and sheep farming areas. Here, a case of 21 year old female with isolated primary hydatid cyst of the kidney is presented, that was evaluated clinically and investigated by x-ray, abdominal ultrasonography, intravenous urography and finally confirmed by histopathology. No complications occurred at peroperative and postoperative periods. The patients were given 3 cycles of Albendazole (10 mg/kg in two divided doses), each for a period of four weeks followed by a 2 week’s rest. The patients are under strict follow-up and no recurrence has been documented yet.