Kidneys, liver, lung. and heart of a 1-year-old male Angora rabbit from a commercial facility were the material of this case. Macroscopically, an amorphous and elastic mass with cysts was observed on the left kidney. The mass weighed 875 grams and was 11 x 12x4.5 cm in dimensions. At the cross section, a tumor with lobular appearance, covering the pelvis and medulla of the kidney was observed, which distinguished from the atrophic kidney tissue via a distinctive capsule. Microscopically, blastemal. epithelial, and stromal areas containing polygonal neoplastic cells with pleomorphic hyperchromatic nuclei and with narrow cytoplasm were noticed. Primitive avascular glomeruluses and undeveloped tubules along with neoplastic cells lined up around vessels were seen in many areas. Occasional cystic forms, mitotic figures. and intensive necroses A ere present in these areas. Stroma was hyperemic and was full of intensive loose and myxomatous vessels. In addition, metaplastic cartilage near capsule was found in the cortex. Immunohistochemically, a strong immunoreactivity was detected with cytokeratin staining only in primitive tubular epithelia and vimentin staining only in blastemal and stromal areas. This is the first report that, a triphasic nephroblastoma (tumour) was diagnosed in an Angora rabbit using histopathological and inimunohistochemical examinations.