Pulmonary arterial hypertension is a vascular disease which is characterized by abnormal proliferation of smooth muscle cells leading to occlusion of pulmonary arterioles, right ventricular hypertrophy and death. Interstitial lung diseases are a diverse group of diseases mainly characterized by inflammation and fibrosis of the pulmonary interstitium. The association of pulmonary hypertension and interstitial lung disease is well known. Increased morbidity and mortality was observed when pulmonary hypertension presents in a patient with interstitial lung disease. The management of pulmonary hypertension in interstitial lung disease is a challenge. We report a case of 60 year old female with severe pulmonary hypertension and interstitial lung disease, who was treated with mycophenolate mofetil along with steroid and antihypertensive drug. Mycophenolate mofetil reduced nocturnal oxygen requirement to 50% after 6 months of treatment and resulted in discontinuation of oxygen therapy after one year of treatment. Profound improvement in pulmonary arterial hypertension and little improvement in interstitial lung disease were observed with no adverse effects.