Chiari malformations are a diverse group of abnormalities of the brain, craniovertebral junction, and the spine. Chiari 0, I, and 1.5 malformations, likely a spectrum of the same malformation with increasing severity, are due to the inadequacy of the para-axial mesoderm, which leads to insufficient development of occipital somites. Chiari II malformation is possibly due to nonclosure of the caudal end of the neuropore, with similar pathogenesis in the rostral end, which causes a Chiari III malformation. There have been significant developments in the understanding of this complex entity owing to insights into the pathogenesis and advancements in imaging modalities and neurosurgical techniques. This article aims to review the different types and pathophysiology of the Chiari malformations, along with a description of the various associated abnormalities. We also highlight the role of ante- and postnatal imaging, with a focus on the newer techniques in the presurgical evaluation, with a brief mention of the surgical procedures and the associated postsurgical complications.