Renal hydatidosis is caused by Echinococcus granulosus and is extremely rare, accounting for 3-4% of the cases of hydatidosis, being the third site after liver and lungs. It generally remains asymptomatic for years and the most frequent symptoms are pain, feeling of flank heaviness, and dysuria. In front of a compatible clinical picture, we studied the diagnostic, therapeutic, and follow-up schemes reviewing the literature. METHODS: We performed an electronic bibliographic search in PubMed (MEDLINE) which MESH terms " Echinococcosis"[MeSH] AND "urinary tract"[MeSH] and bibliographic citations. We perform a review on epidemiology, vital cycle of the parasite and management of patients