Chiari malformation is a congenital malformation characterized with hindbrain hernia. It is generally accepted that it caused by underdevelopment of the occipital somite originating from the paraxial mesoderm. The diagnosis of Chiari malformation is mainly based on MRI. Chiari畸形是以小脑扁桃体下疝畸形为特征的先天性疾患,目前较为公认的理论是起源于胚胎中胚叶轴旁的枕骨原节发育不良所致,其诊断主要依赖于MRI。