Chiari malformation (CM) is primarily characterised by herniation of the cerebellar tonsils through the foramen magnum. Clinically, two main types of CM represent the vast majority of cases: type I (in adults) and type II (in infants). CM may result in neuronal impairment of the brainstem, upper spinal cord and cranial nerves. Part of the afferent and efferet systems and the central respiratory controlling system are located in the cranium-cervical transition and may be damaged in these pathologies, leading to respiratory disorders, such as respiratory failure and death. The ventilatory responses to exogenous and endogenous stimuli, such as responses to hypoxia and hypercapnia, are usually diminished, and apnea may be manifested and detected during sleep, allowing for the diagnosis. This study is a review of the relationship between sleep apnoea and adult CM.