A nine-month-old infant presenting with an isolated oculomotor nerve d
ysfunction was found to have a poorly differentiated neoplasm of the m
esencephalon, an unusual presentation of an unusual tumor. Acquired, i
solated, third cranial nerve palsy in a child poses a challenging diff
erential diagnosis. Although tumor is a rare cause of this finding, fu
ll evaluation for malignancy is warranted in such a case.