Detection of the hypoplasia of the cerebellar vermis is achieved through a cranial magnetic resonance imaging (MRI) scan. The presence of the ‘molar tooth sign’ (MTS) on the MRI scan, a mid- brain hind- brain malformation, confirms this condition and is a key indicator of COACH syndrome. The MTS’s distinguished shape is attributed to the lengthened superior cerebellar peduncles and deepened interpeduncular fossa.[1] To diagnose ataxia, both neurological assessment and physical examination are required.