Hydatid disease is caused by Echinococcus granulosus; a parasitic infestation commonly affecting liver and lung. Isolated renal involvement is rare. Unusual locations, absence of specific diagnostic tests and varied clinical and radiological presentations may pose a diagnostic challenge. High index of suspicion of this disease should be kept for any space-occupying lesion in the kidney. Early pre-operative diagnosis in combination with medical and surgical treatment may prevent dreaded complications. This case is presented here due to its rarity and to highlight the gross, microscopic, and radiological features of isolated renal hydatid disease.
