A 40-year-old woman had a highly pigmented, slightly elevated tumor on the left optic disc. She had no visual disturbance in the left eye. The tumor was stationary for 5 years. At age 45 years, she complained of decreased visual acuity in the right eye. Magnetic resonance imaging showed a right-shifted homogeneous lesion at the tuberculum sellae. Histopathologic study of the excised lesion revealed interlacing bundles of spindle-shaped fibroblast-like cells with whorl formation. We believe that a relationship between melanocytoma of the optic nerve head and a tuberculum sellae meningioma may exist rather than a chance occurrence, as previously suggested by others.