 The paper describes a rare case of a renal composite tumor in a two-year-old girl who has nephroblastoma and renal-cell carcinoma of alveolar-tubular structure with focal expression of pancytokeratin AE1/AE3, cytokeratins 7 and 20, EMA, vimentin, CD10, MELAN A, p 504 S, E-cadherin, most likely, carcinoma with Xp.11.2 translocation.. 