Background:

The renal carcinoma with sarcomatoid differentiation confers a poor prognosis due to its metastatic evolution, less than 1% corresponds to oral mucosa.

Case report:

66 year old female, left kidney tumor, with pathology report clear cell renal carcinoma, with sarcomatoid pattern, gingival tumor in the left maxilla, biopsy with sarcomatoid metastases, received local radiotherapy. It progressed with metastases to the central nervous system, pulmonary, mediastinal and adrenal.

Conclusions:

The clinical presentation of kidney tumors in oral mucosa is rare, the presence of sarcomatoid differentiation implies a therapeutic challenge due to the response to existing systemic therapies, advances in drugs with immunological activity could improve response rates.