Primary squamous cell carcinoma of the kidney is a very rare clinical entity. The lack of characteristic presentation like hematuria, pain and palpable mass causes delay in diagnosis results in locally advanced or metastatic disease at presentation. We report a case of this rare tumor present in right kidney with calculi and hydronephrosis, treated with right nehrectomy. Adjuvant treatment has been tried in form of combination chemotherapy. However, due to aggressive nature of this rare tumor, the prognosis is poor and most of patients develop recurrence or disseminated metastatic disease.
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