目的　提高对窒息性胸廓发育不良的认识。方法　对 2例窒息性胸廓发育不良的临床表现、影像特征进行描述 ,并结合文献分析。结果　 (1)窒息性胸廓发育不良是一种少见的常染色体隐性遗传性骨软骨发育不良性疾病。 (2 )多见于婴儿早期 ,但可迟至青春期 ,临床以胸廓狭小及伴发的呼吸系统异常 ,肾消耗病的进行性加重为主要表现 ,并可有其他系统合并症。 (3 )影像表现特征为胸廓狭长 ,骨盆畸形及肢体畸形。包括 :长骨短、干骺端增宽、掌指骨短粗、锥形骨骺及赘生指。结论　窒息性胸廓发育不良有特征性影像学表现 Objective To raise awareness of asphyxia thorax dysplasia. Methods The clinical manifestations and imaging features of 2 asphyxiated thoracic dysplasia were described and analyzed in combination with the literature. Results (1) Asphyxia thorax dysplasia is a rare autosomal recessive osteochondrodysplasia. (2) Prevalent in early infancy, but late to adolescence, clinically small thoracic and concomitant respiratory system abnormalities, progressive worsening of renal consumptive disease as the main performance, and may have other systemic complications. (3) The imaging features of thoracic stenosis, pelvic deformity and limb deformity. Including: long bones short, metaphyseal widening, metacarpophalangeal, conical epiphyseal and neoplastic means. Conclusion Asphyxia thorax dysplasia has characteristic imaging findings