Pulmonary veno-occlusive disease (VOD) is a rare cause of pulmonary hypertension (HTN) which has been described in association with a variety of clinical conditions. Rapidly progressive occlusion of pulmonary veins and venules develops that is usually fatal. Recently, a number of cases have been reported in patients with malignancies. To date, 33 patients have developed pulmonary VOD in association with a malignancy or after chemotherapy and/or radiation therapy. Two-thirds of the cases occurred following allogeneic bone marrow transplantation (BMT). We describe the first case of pulmonary VOD following autologous BMT and review the experience of pulmonary VOD in patients with malignancies.