Renal Angiomyolipoma (AML) is not commonly seen in the pediatric age group other than patients of tuberous sclerosis where in they can have renal AMLs within the first decade with bilateral in involvement. Diagnosis of renal AML can generally be made by the current radiological modalities in the appropriate clinical setting, but it can be mistaken for other tumors when it is large and has low-fat content. Herein we report a case of giant renal AML that was initially misdiagnosed as a Wilms tumor in a 12-year-old girl.