Pulmonary hypertension is known to occur in Takayasu's aortoarteritis. It may be either due to pulmonary arterial involvement or elevated left ventricular end diastolic pressure, or both. In our case, the cause of pulmonary hypertension was a recently described rare lesion termed pulmonary capillary haemangiomatosis. This entity has a very distinct histopathologic picture. Although 19 cases have been reported in the English literature, this is the first report on pulmonary capillary haemangiomatosis producing pulmonary hypertension in Takayasu's aortoarteritis.