Epitheloid hemangioendothelioma is a vascular tumour with an epitheloid appearance, originating from endothelial cells. Although it is a slow growing tumour, extensive pulmonary involvement, intrathoracic spread, and systemic spread have been documented. We present a case of epitheloid hemangioendothelioma of the lung in a patient with an initial diagnosis made by transthoracic biopsy. The prognosis is unpredictable, with life expectancy ranging from 1 to 20 years. There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported. Our patient underwent pulmonary lobectomy of the right lower lobe and pulmonary wedge resection of the nodule located in the left lower lobe.