Genitourinary echinococcosis is an uncommon cyclo-zoonotic disease. It is caused by Echinococcus species. Kidneys are the common target organs in the genitourinary tract. Clinical presentation is usually non-specific. Pre-operative diagnosis requires a high index of suspicion. It should be considered in differential diagnosis of cystic mass of genitourinary tract, especially in endemic regions. Computed tomography, ultrasonography, and antibody testing aid in diagnosing and classifying the stage of the cyst. Although a number of operative techniques have been described, complete removal of germinal layer with daughter cysts without spillage of viable cyst contents with perioperative medical therapy provides an optimum chance of cure with minimal morbidity. Considering the benign nature of the disease, organ preservation is feasible in majority of the cases. The conventional open surgical treatment has evolved into minimally invasive laparoscopic, endoscopic, and even robotic techniques with concomitant improvement in postoperative and cosmetic outcome. Given the rarity of the disease, no randomized control trial comparing the treatment modalities for genitourinary hydatid exists. Moreover, long-term follow up in many cases is unavailable. Overall, the incidence of local and systemic recurrence is low.