Progressive systemic sclerosis is a chronic sclerotic disease which causes diffuse, increased deposition of extracellular matrix in connective tissue with vascular abnormalities, resulting in tissue hypoxia. Aesthetic and facial dysfunction are followed by important oral and facial manifestation of disturbances such as xerostomia, the lack of saliva in the mouth, and its associated complications. Most clinical manifestations begin with tongue rigidity. The facial skin changes and bone resorption of mandible angle are often reported. Other systemic changes include the involvement of internal organs which leads to serious complications as well as disorders in the cardiac muscle and Raynaud´s phenomenon. The objective of the this paper is to report two cases of systemic sclerosis in patients with oral and facial manifestations of the disease. A brief review of the literature, focusing on deontological alterations is also presented.