The authors report a case of a 20 year old male with ganglioneuroma in the cervical ganglion. The patient was complaining of a mass in the neck on his right-hand side, and neurological examination revealed hypesthesia on the C5 area of the same side. Computed tomography and magnetic resonance imaging scan showed extradural dumbbell-shaped tumor from C3 to C5 levels. A two-staged operation was performed to remove the tumor totally, and a ganglioneuroma was diagnosed. After surgery, he complained of facial weakness and hearing disturbance on the left side. As MRI scan revealed bilateral cerebro-pontine angle tumors, he was diagnosed as having neurofibromatosis-2. This report may represent a rare case of ganglioneuroma with neurofibromatosis-2.