Giant cholesterol cyst (GCC) of the petrous apex is a rare clinical en
tity. This benign cystic lesion can cause neurologic deficits and vasc
ular compromise by persistent growth and progressive bone destruction.
Magnetic resonance imaging studies of GCC show the lesions to be hype
rintense on T-1-weighted sequences with progressively lower signal int
ensities on the first and second echoes of T-2-weighted sequences. The
se findings are relatively specific for GCC, permitting a narrow diffe
rential diagnosis. The goal of surgery is to provide adequate drainage
with the creation of a permanent fistula. The classic approaches to t
hese lesions are the posterior fossa craniotomy and the middle fossa e
xtradural craniotomy. The translabyrinthine approach provides wide exp
osure at the expense of cochlear and vestibular function. The transsph
enoidal approach provides adequate drainage with hearing preservation
and no craniotomy. The endoscopic, endonasal transsphenoidal approach
to a 2.5 cm GCC of the petrous apex accomplished complete drainage wit
h the creation of a fistula. Advances in endoscopic technique and inst
rumentation facilitated the addition of the approach to the surgeon's
armamentarium. In selected cases, this approach provides adequate surg
ical exposure with minimal morbidity.