Arthrogryposis multiplex congenita (AMC) is an uncommon congenital disorder characterized by multiple fixed joint deformities and non-progressive neuromuscular dysfunction. A small fraction of these infants will present with otolaryngologic problems resulting from cranial nerve weakness, muscle dysplasia, or structural dysharmony of the head and neck. The charts of 50 patients with AMC were reviewed to determine the incidence of these findings. A summary of the literature is presented discussing the etiology, pathophysiology, diagnosis and management of this interesting clinical problem.