Teratoid Wilms' tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. We report a case of teratoid Wilms' tumor in a 2-year-old male. Right sided abdominal mass was the presenting complaint. Ultrasonography of the abdomen showed a mass in the right kidney. Histopathological examination revealed blastemal, epithelial, and mesenchymal components along with areas presenting heterologous elements. More than 75% predominance of squamous differentiation with the keratin pearl formation was observed. The patient underwent nephrectomy and was followed post-operatively for 1 year and was normal.