Oculomotor nerve palsies involving only the internal eye muscles are e
xtremely rare. The authors report a 33-year-old female who presented w
ith isolated pupillary and ciliary dysfunction that eventually turned
into a typical third nerve palsy. In 1979 she developed a tonically di
lated pupil with preserved, but reduced accommodation. In 1993 she beg
an to develop diplopia on eccentric gaze direction. The pupil had beco
me dilated and completely immobile and the ciliary muscle was paralyti
c. Eye motility abnormalities were consistent with a mild third nerve
paresis and there was an elevation of the upper eyelid on adduction (p
rimary misdirected regeneration). MRI revealed a parasellar mass lesio
n, probably a neurinoma, arising from the right oculomotor nerve. The
authors decided not to treat this slowly growing tumor.