Hydatidosis is a disease that occurs frequently in certain areas and results from the presence of larvae of Echinococcus Granulosus. Renal involvement is rare, affecting less than 5% of verified cases. Typically asymptomatic for an extended period, renal hydatid cysts only manifest complications before they are diagnosed. There is suspicion based on epidemiological, clinical, radiological and biological information. Clinical presentations can vary, but hydaturia is a pathognomonic feature that appears in 10 to 30% of cases. The improvement of diagnostic accuracy has played a role in this regard. Utilization of ultrasonography is common and both computed tomography and magnetic resonance imaging are useful in verifying the diagnosis. Surgery primarily serves as the basis for treatment. The current standard procedure for preserving the kidney involves removing only the prominent dome through resection. In cases where renal destruction has occurred, total nephrectomy may be necessary. Percutaneous management techniques involving puncture, aspiration, injection and reaspiration should only be considered in highly selective instances. Nevertheless, the efficacy of this technique is still a topic of debate.