Systemic Sclerosis (SSc) is an autoimmune disorder with unknown etiology, which presents with vascular lesions and fibrosis of the skin and internal organs. Cardiac, renal, and pulmonary involvements are major causes of death in systemic sclerosis. Although a major cause of scleroderma deaths is cardiac failure, scleroderma rarely causes valvular disease. We report the case of a 48-year-old woman with severe mitral stenosis who was diagnosed with scleroderma 6 months before. Echocardiographic assessments had revealed no clinical manifestation of heart involvement in her previous visits. We used cardiac surgical treatment with successful outcome. The present case confirms the data from the currently available literature, indicating that mitral valve involvement in systemic sclerosis is a rare occurrence. Awareness of this uncommon association and adequate management to prevent complications of the underlying disease are prerequisites for successful mitral valve repair or replacement in such patients.