Neuroimaging techniques have refined the characterization of neural structures involved in the regulation of normal sleep-wake cycle in healthy humans. Yet brain imaging studies in patients with sleep disorders still remain scarce. In narcoleptic patients, structural and functional brain imaging studies have suggested the involvement of the hypothalamus in the pathophysiology of narcolepsy, plausibly consistent with an impairment of the hypocretin-orexin system. Some recent studies have further suggested that cataplexy, a key feature of the narcoleptic syndrome, might result from a dysfunction of the hypothalamus and its interactions with limbic structures. Other neuroimaging studies have focused on the assessment of neurotransmission and the effects of pharmacological treatment in narcoleptic patients. However, the neural correlates of some main symptoms of narcolepsy, such as sleep attacks, hypnagogic/hypnopompic hallucinations and sleep paralysis, are still unknown. In addition, the description of brain activity patterns during sleep in narcoleptic patients needs further investigation. Neuroimaging has proven to be a valuable tool for the study of sleep regulation and sleep disorders; its future developments will undoubtedly improve our understanding of the neural mechanisms underlying narcolepsy with cataplexy.