Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal neoplasm, which occurs most commonly in uterus and gastrointestinal tract. PEComa with perirenal manifestation is an extremely rare entity. To the best of our knowledge, only four cases have been reported up to now. In this case, we reported a patient with both a pulmonary mass and a perirenal mass. Two resections were performed successively and postoperative pathology suggested pulmonary micro invasive adenocarcinoma (MIA) and perirenal PEComa. This is the first case of perirenal PEComa with pulmonary MIA. Combining the present case and prior literature, we summarized the crucial role of immunohistochemistry in the diagnosis and consider that complete operation might be conducive to patients with perirenal PEComa that presents a benign phenotype, regardless of complications with other tumors.