Tumor lysis syndrome is a set of life threatening complication that can arise from treatment of high tumor burden, drug sensitive, and rapidly proliferating neoplasm particularly of hematological origin. It is rarely described in patients with solid tumors. We report the first case of tumor lysis syndrome in a man with metastatic renal pelvic transitional cell carcinoma after gemcitabine treatment. Despite aggressive therapy, he died 2 weeks after TLS was diagnosed. Our experience demonstrates that administration of gemcitabine for metastatic renal pelvic transitional cell carcinoma may induce acute tumor lysis syndrome, which necessitates frequent laboratory monitoring and prompt initiation of appropriate therapeutic measures.