This case series examined preoperative findings and the surgical, anesthetic, and postoperative management of 6 patients with congenital myopathies (CMs) and congenital muscular dystrophies (CMDs) treated at a tertiary medical institution with orthognathic surgery over 15 years to describe pertinent considerations for performing orthognathic surgery in these complex patients. Patients with CMs or CMDs often have characteristic dentofacial malocclusions that contribute to functional problems with feeding and drooling and psychosocial problems. Orthognathic surgery, usually bimaxillary, can be judiciously considered in these patients; these procedures typically require multidisciplinary pre- and postoperative evaluation and care over lengthy hospital stays with a high risk of respiratory complications that bear consideration in treatment planning.