Echinococcosis is endemic in many parts of the world. Although echinococcosis can be present in all parts of the human body, urinary tract involvement develops in only 2-4% of all cases, and isolated renal cysts are extremely rare. There are no specific clinical symptoms or signs that will reliably confirm the diagnosis of renal echinococcosis. Routine blood tests are generally normal except for eosinophilia, which is found in only 20% of the cases. Radiological studies have a more important place in the preoperative diagnosis of renal hydatic disease. However, there is no specific sign on plain urography or intravenous urography, and ultrasound or computed tomography cannot always show a echinococcosis as a specific lesion. Echinococcosis should be included in the differential diagnosis of cystic lesions in solid organs or other anatomic sites, especially in endemic countries. We presented a patient with isolated giant renal cystic echinococcosis mimicking a complicated cyst who was treated successfully with nephrectomy.