Arthrogryposis multiplex congenita (AMC) consists of complex congenital anomalies characterized by multiple contractures. Anesthetic management of these patients requires special care: as this disease often progresses until dysfunction of multiple organ systems occur, it may have an impact on the anesthetic management. Here, we report a case of AMC undergoing urgent surgery for open tibia fracture who had difficult airway management because of limited mouth opening. The anesthetic management of this patient is represented in light of the literature. © 2011 Japanese Society of Anesthesiologists.