Arthrogryposis multiplex congenita (AMC) is a rare congenital disorder characterized by multiple fixed-joint deformities of the upper and lower extremities. Those afflicted with the more common neurogenic form of AMC can have otolaryngologic manifestations. Here, a case of AMC with severe otolaryngologic manifestations is reported. The clinically relevant findings included an expressionless face, micrognathia, poor suck reflex, high arched palate, and an omega-shaped epiglottis, but otherwise normal larynx, trachea, and esophagus by endoscopic examination. Airway compromise, achalasia, aspiration pneumonitis, and poor nutrition were constant problems. Tracheostomy and gastrostomy were subsequently placed, and the patient's clinical course eventually improved. Early involvement of the otolaryngologist and placement of a tracheostomy and gastrostomy are recommended in those patients with severe otolaryngologic manifestations of AMC.