Schwannomas of the abducens nerve are extremely rare tumors affecting cavernous, cisternal or both segments of sixth cranial nerve. Clinical features and neuroradiological imagery are frequently insufficient to reach an accurate pre-operative diagnosis. We report a patient with a cystic tumor with ring-like contrast enhancement at the right anterior pontomesencephalic junction. Radical excision was performed via anterior transpetrosal approach and showed an extrinsic tumor originating from the sixth nerve. A postoperative sixth nerve palsy had disappeared completely 9 months after the surgery. The correct diagnosis of an abducens nerve schwannoma is established by the intraoperative finding of a tumor attachment to the sixth nerve and by histopathological analysis. The various differential diagnoses, the clinical and radiological features of this diagnosis and management are issues discussed in this illustrated review.
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