Squamous cell carcinoma (SCC) of the kidney is a rare and fatal clinical entity. It is usually aggressive with generalized disseminated disease upon presentation. Here we report the case of 61 years old male patient, with no previous history of kidney stones or other medical problems, who presented for dyspnea, hemoptysis, left flank pain and hematuria. He was diagnosed with stage IV undifferentiated SCC of the left kidney. He had a dramatic clinical evolution, with rapid progression, leading to deterioration and death. Renal squamous cell carcinoma remains an unusual, unsuspected diagnosis of kidney tumors with a poor prognosis.
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