Primary sarcomas of the kidney are a rare entity and it counts for 1-3% of malignant neoplasms. The most frequent histologic types are leiomyosarcoma and liposarcoma. The primary pleomorphic undifferentiated sarcoma of kidney is a rare variant and it has a poor prognosis, with around 60 cases described in the literature up to date. We present a 52-year-old female case with high renal tumor, treated surgically whose histological diagnosis confirmed by immunohistochemistry was undifferentiated pleomorphic sarcoma, giant cell variant.