Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension.The diagnosis of PVOD/PCH can be established by the combination of clinical features,physical examination,radiological findings,lung function,bronchoscopy and other resources.There is no established medical therapy for PVOD/PCH,and the only curative therapy for PVOD/PCH is lung transplantation.A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital.Combining the characteristics for this case with the relevant literature,we summarized the epidemiology,etiology,diagnosis and treatment for the disease to raise doctors' awareness for this rare disease.