A fatal case of pulmonary venoocclusive disease in a 4-year-old boy is reported, with emphasis on the radiologic features of this condition. The disease is characterized by fibrous intimal obliteration of the lumina of small pulmonary veins and venules leading to severe secondary pulmonary arterial hypertension. The clinical picture is usually indistinguishable from that of other forms of pulmonary arterial hypertnesion, but radiologic features of postcapillary pulmonary hypertension, in conjunction with a normal venoocclusive disease. The diagnosis is definitively established by lung biopsy.