Hydatid disease is a parasitic disease caused byEchinococcus granulosusorEchinococcus multilocularis. It is still a serious public health problem in endemic regions such as the Mediterranean basin. Since the complaints caused by the cysts are non-specific and routine laboratory tests do not always yield positive results, diagnosis may be difficult. While liver involvement is present in 70% of cases, larvae escaping from the filtration of the liver cause pulmonary disease in 25% of cases. Although the prevalence of kidney involvement in all hydatid cysts is approximately 2-4%, and isolated kidney involvement is extremely rare at 1.9%. In this case report, we present an extremely rare pediatric case of isolated renal hydatid cyst, the diagnosis of which was somewhat delayed.