Epithelioid hemangioendothelioma (EH) is a rate pulmonary vascular malignancy. Clinically, EH has been considered as an indolent, generally non-aggressive tumor. We report a case of EH which was confirmed by open lung biopsy. A 34-year-old woman was admitted for further evaluation of multiple small (less than 2cm in size) nodules, incidentally detected on screening chest radiograph. The chest CT showed multiple, relatively well-marginated, variable sized nodules at both whole lung. Transbronchial lung biopsy and transthoracic needle aspiration were nondiagnostic and open lung biopsy was performed from right middle lobe of lung. On light-microscopic examination, the nodules were composed of a poorly cellular hyaline core and a more cellular peripheral zone which extended into air space in a micropolypoid fashion and obliterated blood vessels. The tumor cells at the peripheral zone had intracytoplasmic vacuoles which suggested primitive, vascular differentiation. Immuno-histochemical study revealed the cellular area which gave positive reaction to factor VIII-related antigen. She received no specific therapy after open lung biopsy and chest X-ray films had showed no change for about two years.