Scleroderma, or progressive systemic sclerosis (PSS), an autoimmune rheumatic condition affecting the connective tissues, has a profound impact on oral health. Common orofacial findings include xerostomia, gastroesophageal reflux disease and limited mouth opening. This review article describes scleroderma, or PSS, and its various manifestations. The features of CREST syndrome and morphea are reviewed. Concerns relevant to the prevention of dental disease and the safe delivery of dental care in this group of challenging patients are emphasized.