Echinococcus granulosus, also known as the dog tapeworm, causes echinococcosis or hydatid disease in humans. It is an anthropozoonotic and non-endemic disease. Hydatid cysts are most commonly found in the liver and lungs, but can occur in any other organ, including the brain, kidneys, bones, and peritoneal cavity. Isolated renal hydatidosis is an extremely rare condition, accounting for only 2-4% of all cases of hydatidosis, with its occurrence in children being even rarer. We are reporting a rare case of isolated renal hydatidosis in a 12-year-old boy.