The craniofacial manifestations, together with teleradiographic and cephalometric findings, of Steinert's dystrophy were examined based on a review of the literature and the clinical features of several patients with myotonic dystrophy leading to the diagnosis of Steinert's disease. The prevalence of the functional impairments due to facial deformation varies with the severity of the disease, suggesting early interceptive treatment supported by orthopaedic care and rehabilitation therapy of orofacial functions. Surgery involves bimaxillary osteotomy which can improve mastication and facial morphology. Operative risks should be carefully identified with adequate explorations before surgery.