Hydatid disease constitutes an endemic zoonosis caused by different species of the cestode Echinococcus. The main organs affected are the liver and the lungs. Echinococcosis of the urinary tract is an extremely rare medical condition, met in 2-4% of cases. This pathologic entity has to be included in the differential diagnosis of occupying lesions of the urinary tract at any age, especially among patients in endemic areas. Meticulous laboratory and imaging control when taking into consideration the suspicion for the disease, can guarantee an accurate diagnosis and successful outcome.