Preoperative diagnosis of renal hydatid disease is difficult even in an endemic zone. Imaging studies are suggestive but usually inconclusive, and the differential diagnosis with a renal tumor or complicated cyst may not be made without surgery. Renal sparing surgery is possible in a significant proportion of cases, particularly when preoperative diagnosis has been considered. Significant surgical morbidity can be expected, and the risk of anaphylaxis and hydatid seeding, although low, should not be overlooked.