Teratoid Wilms' tumor (WT) is a rare variant of nephroblastoma which had been only reported in pediatric patients. Herein, we report a 32-years-old female patient with teratoid WT with predominant heterologous neuroepithelial component of the right kidney. Histologically, the tumor showed the typical histological features of a teratoid WT formed of triphasic pattern of blastema, epithelium and stroma with heterologous elements which comprised more than 50% of the tumor area. The heterologous elements were mainly composed of neuroepithelial tissue including ganglion cells, differentiating neuroblasts, true and pseudorosettes, ganglioneuromatous differentiation, schwannianstroma, abundant neuropils admixed with mature types of epithelium and rhabdomyoblasts. This was the second reported case of adult teratoid WT with extensive neuroepithelial differentiation.