Pulmonary capillary hemangiomatosis (PCH) is a rare idiopathic lung disorder that occurs in young patients and leads to pulmonary hypertension (PH). It is difficult to diagnosis in the early stage and is often mistaken for primary PH; in almost all cases of PCH, the correct diagnosis is not made until autopsy. In the present case of PCH, the patient had severe pulmonary hypertension and died of respiratory failure. Pathologically, PCH is characterized by proliferation of benign thin-walled capillary sized blood vessels in the lung parenchyma.