Cystic hydatid disease is a parasitic disease caused by the larvae of the small tapeworm Echinococcus granulosus. It is still a serious public health problem in endemic regions such as the Mediterranean basin, especially in the Balkans. Usually, the complaints caused by the cysts are non-specific and there are rarely abnormalities in routine laboratory tests. The most common is the involvement of the liver. The frequency of isolated kidney involvement, especially in a child, is uncommon. We describe a rare pediatric case of an isolated renal hydatid cyst presenting with a urinary tract infection-like clinical presentation, leading to misdiagnosis and delayed treatment.