Human echinococcosis (hydatid disease) is highly endemic in Morocco and reemerging as a significant public health problem in this country. It is a cyclozoonotic infection introduced by Echinococcus granulosus, that can invade any organ in the human body. The liver is the mostly affected organ, which is the primary site of human infestation, followed by lungs. Renal involvement by hydatid disease is commonly secondary, occurring owing to primary cyst rupture or disseminated disease. Primary renal localization, often presented as solitary cysts with no visceral involvement, is uncommon even in endemic zone. Herein, a rare cause of renal mass due to an isolated hydatid cyst in a 56-year-old woman who presented with ambiguous left flank pain for the last 10 years. Findings imaging did not reveal any other localizations of hydatid disease. For this scolicidal effect and to prevent peritoneal seeding, the patient was placed on albendazole 400 mg. Three weeks later, the surgery was performed successfully with a left total nephrectomy.