We report a case of idiopathic pulmonary veno-occlusive disease (PVOD). The patient experienced progressively worsening dyspnea. Heart catheterization revealed severe pulmonary hypertension. High-resolution computed tomography (HRCT) showed diffuse, poorly identified centrilobular ground-glass opacities. Surgical lung biopsy led to the diagnosis of PVOD. A microscopic examination revealed occlusions of pulmonary veins and venules over a wide area with prominent loop-like capillary dilatations. These pathological findings may be correlated with the radiological characteristics of HRCT in this case.