Epithelioid hemangioendothelioma of the lung, therefore labeled as IntraVascular BronchioAlveolar Tumor (IVBAT), is a tumor of endothelial origin; its presentation with multiple pulmonary nodules radiographically suggests metastatic disease. We describe here an epithelioid hemangioendothelioma with an exclusively myxoid pattern. The tumor was composed of nodules extending in a polypoid fashion from the alveolar septa. The cells within the tumor were epithelial-like and contained cytoplasmic lacunae positive for anti-FVIII, CD31 and CD34 antibodies. According to usual criteria, it was considered as a tumor of low grade malignancy, without vascular or bronchiolar extension or cytonuclear atypia and mitosis; the prognosis, difficult to determine, depends on locoregional tumor progression.