Objective The objective of this study was to summize the experience of management of airway in scoliosis patients associated with arthrogryposis multiplex congentia (AMC). Methods The medical records of twenty scoliosis patients associated with AMC undergoing spinal corrective surgery in our hospital were retrospectively reviewed. These data were noted as following: patients’ demographics, the degree of Cobb angle before surgery, the grade of Mallampati, the results of pulmonary function tests, whether was difficult airway or not, the special instrument for intubation, whether was admitted to ICU or not after surgery, and the time from end of surgery to tracheal extubation. Results There were twelve male patients and eight female patients in our study. The age of patients averaged 14.9±3.7 years, ranging from 9 to 24 years. The median degree of preoperative Cobb angle was 90 degrees, with the maximum Cobb angle of 147 degrees. The cases of MallampatiⅠ, Ⅱ, Ⅲ, and Ⅳ grade was 3 (15%), 9 (45%), 5 (25%), and 3 (15%), respectively. The cases of mild, moderate and severe preoperative pulmonary dysfunction was 1 (5%), 4 (20%) and 15 (75%), respectively. Five patients with anticipated difficult airway were successfully intubated via fiber bronchoscope. After surgery, seven patients with difficult tracheal extubation caused by lung ventilation dysfunction were admitted to ICU to monitor vital signs closely. The time from end of surgery to tracheal extubation varied greatly, ranging 10 minutes to 1115 minutes. Conclusions The patient’s airway should be thoroughly examined by the attending anesthesiologists during preoperative interview, whose attention should be focused on assessing difficult airway, especially for difficult intubation. The anesthetists should make well preparations for difficult airway, especially for difficult intubation, including emergency drugs, and special instruments for intubation.