Neurilemmomatosis is a clinical entity consisting of multiple cutaneous neurilemmomas, central nervous system tumors, and neurologic disorders. Since Shishiba et al first described the disorder in 1984, several cases have been reported. We report a Korean case of neurilemmomatosis showing multiple neurilemmomas of the skin and spinal cord, with associated motor and sonsory nerve disturbance. Histologic, immunohistopathologic and electron microscopic studies confirmed the diagnosis.