A 62-year-old, obese woman, smoking 10 pack/year was admitted to the National Tuberculosis and Lung Diseases Research Institute to diagnose small, round opacities revealed by routine chest X-ray examination. These lesions had been observed for 5 years. The patient had been treated for psoriasis, hypertension, and insulin-independent diabetes. On admission she was in good condition, complaining of a slight productive cough as well as intermittent osteoarticular pain. Physical examination revealed cutaneous psoriatic lesions, slight edema of the lower limbs, and clubbed fingers. Tuberculin test was positive. Chest Computer Tomography scanning showed partially calcified nodules (up to 1 cm in diameter) located in the middle and base areas of both lungs. No evidence of hilar nor mediastinal lymph node enlargement was seen. Lung specimens displayed intraalveolar and intravascular growth of neoplastic cells. Immunohistochemical expression of Factor VIII, CD31 and CD34 antigens was present. Pulmonary epithelioid haemangioendothelioma was diagnosed. After 6 months of observation, progression of the disease was shown. Interferon alpha treatment was introduced. During the therapy, a slight regression of pulmonary changes was noticed and since then stabilization of the disease was observed.