A unique, deep-seated, benign, glandular peripheral nerve sheath tumor (PNS
T) of the cerebellopontine angle is described. The tumor arose from the sev
enth and eighth cranial nerve complex in a 15-year-old boy without neurofib
romatosis. Histologically, benign glands were embedded in a bland spindle c
ell stroma. The epithelial cells were immunoreactive for CAM 5.2, and focal
ly for chromogranin. The spindle cells were positive for S100P. The benign
glandular PNST unassociated with neurofibromatosis is a controversial entit
y. The superficial location of most reported cases has made it difficult to
exclude entrapped adnexae as a source for the glands. This tumor was separ
ate from the internal auditory meatus on MRI scan, the most likely source o
f entrapped glands at this site. This case is the first report of a deep-se
ated, benign, glandular peripheral nerve sheath tumor. It suggests that gla
ndular differentiation in PNSTs, while unusual, is not synonymous with neur
ofibromatosis or malignancy.