Epithelioid haemangioendotheliomas are rare tumours of endothelial origin. They can develop in any tissue but occur principally in the lung and liver. Their usual course is a slow progression, so that they can be treated by surgery. In aggressive forms, no treatment has proved efficient to date. This study, describes a case of bilateral pleural epithelioid haemangioendothelioma that extended to the peritoneum. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. After six courses of carboplatine plus etoposide, a complete response was obtained. The complete remission is still lasting at 18 months after the diagnosis and the patient is healthy.