Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor that occurs among young women and typically presents as bilateral multiple nodules. In the present report, we describe an uncommon case of PEH presented as a single cavitary nodule in a 33-yr-old asymptomatic man. This is the first case of PEH presented as a single cavitary nodule in the English literature. Three years of the follow-up without treatment was performed. Overall histologic findings were accord with conventional PEH, but some atypical features such as, increased mitotic activity (mean; two per ten high power fields), necrosis, spindling, and pleural and vascular invasion were recognized. Immunohistochemically, the tumor cells were positive for CD34. This report may contribute to the data on clinical findings and natural history of this rare tumor.