In a 66-year-old patient morphologic features of a pulmonary veno-occlusive disease were found. Besides, a striking foreign body giant cell reaction with phagocytosis of altered elastic tissue in totally or partially occluded venous blood vessels resembled the so-called pulmonary sideroelastosis, an entity originally described by Ceelen. In this case report, a possible pathogenetic relation between sideroelastosis pulmonum and pulmonary veno-occlusive disease is discussed.