Two cases of trochlear nerve neurinoma without Recklinghausen's disease are described. A 60-year-old male had a left-side sensory disturbance and a hemiparesis. Computed tomography (CT) scan and magnetic resonance image (MRI) showed a cystic enhancing mass in the right tentorial incisura. A 57-year-old male had a gait disturbance and a left-side facial numbness. CT scan and MRI showed a solitary enhancing mass in the right tentorial incisura. These tumors were subtotally removed, and the intraoperative diagnosis of two patients was trochlear nerve neurinoma. Unique clinical manifestations of trochlear nerve neurinoma are discussed with a brief review of 11 cases reported in the literature.