This report describes a 7 year old girl who presented with scoliosis and a small right hemithorax. Findings on spine roentgenographs were pleural thickening on the right and parenchymal densities. Computed tomography of the chest and abdomen revealed multiple pulmonary nodules and two hypervascular lesions within the liver. The diagnosis established by lung biopsy was intravascular bronchioloalveolar tumor (epithelioid hemangioendothelioma of the lung). This is the youngest reported case of this rare tumor.