Arthrogryposis multiplex congenita syndrome, characterized by multiple congenital joint contractures, is a manifestation of a neurogenic or myopathic disorder of unknown etiology. An accurate diagnosis is critical in management strategy. Muscle biopsy is usually the most important diagnostic procedure. Once the diagnosis is established, a team approach to comprehensive care is instituted, and the child is assisted in achieving maximum cognitive, physical, and social development. Physicians, nurse clinicians, therapists, social workers, and others constitute the team and work in coordination to develop an individual habilitation plan. The treatment plan is continually updated and modified as needed. Because the musculoskeletal deformities are generally the most prominent abnormality, the orthopedist usually has the major role in management. The orthopedist may serve as coordinator of the comprehensive care team and therefore must have a fundamental understanding of and global approach to management.