Subjects affected with inherited disorders, of the connective tissue make up an important population, carrying high risks as to distinct aspects of oral health and dental treatment. These generalized conditions may produce serious clinical symptoms in different orofacial structures, which have to be dealt with, or anticipated, when considering dental treatment. The most prevalent disorders result from deficiency of Type I collagen, an important extracellular matrix protein regulating both the structural and mechanical properties of most of the orofacial tissues. Recurrent jaw fractures, an increased liability for development of temporomandibular disorders, periodontal disease and mucosal fragility, an abnormal tooth color and/or shape, and pulp obliteration may feature as major clinical manifestations of the respective disorders. Deficiency of fibrillin, a protein providing soft tissues with elastic capacities, may produce a long face with a high and narrow palate, an increased liability for the developnet of temporomandibular disorders and periodontal disease, and root dsyplasia. Whenever present, these manifestations/risk factors have to be integrated in dental treatment strategies. In cases with high risk for cardiovascular complications, specific preventive measures, such as cardiac output monitoring and the administration of appropriate local anesthetics, have to be taken before starting any invase dental treatment. The present paper aims to provide the practitioner with an appreciation of the most prevalent inherited disorders of the connective tissue with their respective genetics, molecular aspects of pathology, medical and oral manifestations, and guidelines for dental treatment.