Tubulocystic carcinoma of the kidney is a recently reported rare entity, with very few reports in the literature. It has always been described as a low-grade malignancy with favorable prognosis. We report the case of 33-year-old man who presented with back pain. Investigations revealed multiple bony metastases and a large, solid, cystic left renal tumor. Decompression of the spine followed by left radical nephrectomy was done. Final pathologic diagnosis was tubulocystic carcinoma of the kidney with sarcomatoid features. Unfortunately, he presented after 2 months with intestinal obstruction, and an exploratory laparotomy revealed multiple peritoneal metastases with ascites.