A rare case of trochlear nerve neurinoma is described. Including this case, the number of reported intracranial tumors arising from the sheaths of the third, fourth, and sixth cranial nerves is 38. By site and relationship to the nerve segment, they fall into three groups: cisternal, cisternocavernous, and cavernous. In cisternal tumors of the third and sixth nerves, paresis of the nerve hosting the tumor is the unique nerve deficit; by contrast, in those of the fourth nerve, paresis of the trochlear nerve can be absent and that of the third nerve present. In the latter tumors, a peculiar ataxic hemiparesis syndrome is produced by midbrain compression. Cisternocavernous neurinomas often cause symptoms of intracranial hypertension, while cavernous neurinomas bring about two clinical features: paresis of one or more nerves of the cavernous sinus and a clinicoradiological orbital apex syndrome. At surgery, generally cisternal neurinomas are totally removed and the nerve source of the tumor identified; in cisternocavernous and cavernous neurinomas, total removal of tumor and identification of the parent nerve have been reported in only half of the cases. In the majority of parasellar neurinomas, clinical differences can be found between those arising from the nerves governing eye movement and those arising from the gasserian ganglion.