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Epithelioid hemangioendothelioma (EHE), a very uncommon pleural tumor, was diagnosed in a 61-year-old man who had suffered work-related exposure to asbestos. The patient complained of chest pain and weight loss and was diagnosed with pleural effusion, in which there was a predominance of lymphocytes but no malignant cells. Pleural needle biopsy disclosed only a non-specific inflammatory process, with areas of myxoid tissue. Video thoracoscopy revealed diffuse nodules in the parietal and visceral pleura. The nodule biopsy revealed a mesenchymal neoplasm and focal areas similar to those seen in the first biopsy. The immunohistochemical study showed the presence of the vascular markers CD 31, CD 34 and VIII factor, characteristic of tumors of vascular origin. The tumor was not responsive to cisplatin or etoposide, and the patient died 3 months after being diagnosed