Nephrogenic adenofibroma is a benign renal tumor in children and young adults described by Hennigar and Beckwith in 1992. Seven cases have been described, and we report the first case in an 11-month-old child, in good health, revealed by a macroscopic hematuria. Nephrogenic adenofibroma is an unusual tumor, which was difficult to distinguish from nephroblastoma and mesoblastic nephroma. Beckwith makes a distinction between this principal differential diagnosis in child renal tumors based upon morphologic and immunohistochemical patterns. In our observation, the diagnosis remained difficult and needed several reviews of our case. Beckwith proposed the final diagnosis: nephrogenic adenofibroma with stromal predominance. The prognosis is excellent and no treatment is indicated. A FISH analysis of the tumor cells found a trisomy 11. Trisomy 11 has been reported in mesoblastic nephroma as the most frequent chromosomal abnormality. This finding in tumor cells provides an argument for excluding the diagnosis of nephroblastoma but can not clarify the difference between nephrogenic adenofibroma and mesoblastic nephroma.