PEComa (clear cell "sugar" tumor) of the lung is a rare benign tumor of the lung probably arising from the perivascular epithelioid cells (PECs). We report a case of pulmonary PEComa arising from the periphery of the right lobe of a 64-year-old male. To our knowledge, this is the second case in the English literature diagnosed by fine needle aspiration biopsy. In this case report, the clinical, cytologic and immunohistochemical features clear cell "sugar" tumor of the lung are discussed and compared with the previously published literature. The differential diagnosis and methods for distinguishing the various clear cells lesions in the lung are discussed.