Narcolepsy associated with localized brain lesions is rare, and few re
ports of well-documented cases have been published. We describe the ca
se of a 20-year-old male (HLA DQw1 negative) who fulfilled clinical an
d polygraphic criteria of symptomatic narcolepsy. Narcolepsy in this p
atient was associated with an arteriovenous malformation involving the
structures around the third ventricle. Clinical symptoms improved aft
er embolization and radiosurgery. These findings support the hypothesi
s that lesions in the vicinity of the third ventricle can cause sympto
matic narcolepsy.