Systemic sclerosis is rare autoimmune connective tissue disorder often localized to the skin. Some occasion; it spread to heart, gastrointestinal system, lung and kidney. The cardiac structures like valve, coronary artery and conduction systems are mostly affected. Systemic sclerosis patients have associated pulmonary hypertension, restrictive pulmonary function, thick skin with ulcers and dysrhythmias. We discuss the management of a 39 year female diagnosed case of systemic sclerosis presented for double valve replacement. She had previous mitral valve commisurotomy 25 yrs back, past episode of stroke, restricted mouth opening resulting in difficult tracheal intubation and restrictive lung disease. The case report described the meticulous medical and surgical management for double valve replacement andsuccessful intubation with fibreoptic bronchoscope. 

Keywords: Systemic Sclerosis; Restrictive Mouth Opening; Cardiac Surgery; Double Valve Replacement.