Perivascular epithelioid cell tumors (PEComas) are made up of cells with distinctive morphologic, immunohistochemical, ultrastructural and genetic features. They may arise in various locations and are usually benign. Malignant PEComas are rare and associated with an aggressive clinical course and metastatic spread. We present a case of a 56 years old healthy patient who presented with right flank pain and a large mass located at the medial aspect of the right kidney. The mass ruptured during laparoscopic removal. The tumor was eventually labeled as an epitheloid malignant neoplasm most suggestive of malignant PEComa. The patient demonstrated during follow up both local and systemic metastasis that are unresponsive to this point to mTOR inhibition with various agents.