Renal epithelial tumours that do not fit into any of the defined entities in the 2016 World Health Organization classification of renal cell cancer (RCC) are diagnosed as renal cell carcinoma, unclassified (RCCU). This category may include tumours with unique morphological patterns that fall outside the known categories, have overlapping features of known subtypes or are pure sarcomatoid tumours demonstrated to be of epithelial origin. We report a 65-year female who presented to us with hematuria and flank pain of 2 months duration. On evaluation, she was diagnosed with right renal tumor. On surgical exploration, the renal mass was firmly attached to lateral wall of inferior venacava (IVC), requiring local excision of lateral IVC wall with primary repair. Histopathological report was suggestive of double morphological variant of renal cell cancer consisting of sarcomatoid (75%) and rhabdoid (25%) differentiation. Patient had early massive recurrence of cancer at local site at 6 weeks follow up and succumbed during the hospital stay.

Keywords: Epithelial tumors; Sarcomatoid; Radical nephrectomy; Recurrence rhabdoid