PURPOSE: To evaluate ophthalmologic findings and long-term course in patients with neurofibromatosis type 2 (NF2). DESIGN: Retrospective observational case series. METHODS: SETTING: Single-center institutional practice. STUDY POPULATION: Thirty referred patients with NF2 were enrolled from 1991 to 2003 and underwent at least one thorough neuroophthalmologic examination. Follow-up of 23 patients ranged from 4.3 to 12.5 years. MAIN OUTCOME MEASURES: Visual function, structural ocular abnormalities, onset and type of presenting NF2-related symptoms, and number of central nervous system tumors. RESULTS: Initial symptoms for patients with early-onset NF2 mostly comprised ophthalmologic symptoms (n = 7) and lower motor neuron extremity weakness (n = 6), as opposed to eighth nerve impairment (n = 11) in late disease onset. NF2-specific ocular findings were noted in 83% of all patients (94% childhood onset; 67% adult onset): 67% had cataracts, 40% epiretinal membranes, 3% hamartoma, 13% disk gliomas, and 27% optic nerve sheath meningiomas. Only 14% in the childhood-onset group-as opposed to 78% in the adult-onset group-sustained visual acuity of 1.0 in both eyes at final examination. Significantly more patients with early onset of symptoms developed multiple central nervous system tumors (P = .004) and showed a higher amount of NF2-specific findings (P = .015). CONCLUSIONS: Initial manifestations of NF2 differ between children and adults. NF2-specific ophthalmologic findings can help establish the diagnosis. Symptom onset at a young age is clearly a risk factor for marked disease progression. These patients should be carefully followed because survival rates have increased, and vision becomes increasingly important as the disease progresses.