Background: Perivascular epithelioid cell tumor (PEComa) is extremely rare neoplasm, especially for the pulmonary localization. Clear-cell tumor of the lung, arising from perivascular epithelioid cells, has mainly asymptomatic and benign course. The diagnosis is confirmed on the immunohistochemical examination. The tumors are usually benign, but there are some sarcoma-like masses. Treatment of choice is a surgical resection. Case Report: We report a case of benign PEComa in a 64-year old non-smoking female, accidentally found on a chest X-Ray. VATS anatomic segmentectomy of basal segments was performed. The immunohistochemical examination showed typical features of PEComa. Conclusion: PEComa should be considered in the differential diagnosis of pulmonary nodules. Surgical resection is a treatment of choice followed by immunostaining.