Background: Systemic sclerosis is an inflammatory fibrotic disease that results in deposition of   excessive extracellular matrix in the skin and several visceral organs including the lungs, heart,     kidneys, and gastrointestinal tract. The peak incidence is between the ages of 20 and 60 and is about   three times more common in women than men. Pulmonary hypertension and pulmonary fibrosis can     be idiopathic or may be due to other diseases, systemic sclerosis can also lead to development of both.

Case Presentation: A 50 years old female presented with progressive breathlessness, chest pain, easy fatigability, dysphagia, short mouth opening and lower limb swelling. On examination patient had sinus tachycardia, raised JVP, pedal odema , loud P2 with pan systolic murmur and bilateral velcro crepitations.

Conclusion: This case illustrates the presentation of pulmonary hypertension and pulmonary fibrosis in systemic sclerosis.