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Background Primary nerve sheath tumor of cranial nerve is a rare intracranial space occupying lesion that most commonly involves vestibular nerve. Oculomotor nerve schwannoma, without neurofibromatosis, is an extremely uncommon entity especially in children. Though any cranial nerve can be involved by schwannoma except I and II nerve as these cranial nerves lack a Schwann cell sheath. Case presentation We herein report a 14-year-old boy presenting with an oculomotor schwannoma in the absence of features of neurofibromatosis, manifesting as progressive diplopia, ptosis, and blurring of vision. Conclusions Most similar previous case reports in literature reported oculomotor schwannoma in adults, unlike our case. The detection rate of such rare lesions has, however, increased in the last two decades due to neuroimaging advances.

Cisternal oculomotor schwannoma: a rare cause of anisocoria with ptosis in a child