A 5-year-old girl presented with a history of bilateral reflux nephropathy and a left kidney mass, suspicious of Wilms tumor on imaging studies. She underwent radical nephrectomy according to the National Wilms Tumor Study protocol. The pathology report was consistent with metanephric adenofibroma (MAF), for which radical nephrectomy was considered curative. MAF is a benign, uncommon tumor, which is frequently confused with Wilms tumor on diagnostic imaging; histopathologic studies reveal the diagnosis. The diagnostic and management approach to MAF is discussed.