A case of neurothekeoma in a 52-year-old woman is reported. The tumor developed on the medial aspect of the right nostril as a well-demarcated, dome-shaped, erythematous nodule of rubbery consistency. Microscopically, it consisted of numerous nests and cords of spindle-shaped cells in the dermis. Some of the tumor cell nests appeared epithelial-like, while the other areas showed a myxomatous appearence with various amount of mucinous matrix in the intercellular space. Neurothekeoma is a benign cutaneous tumor, and is considered to be of schwann cell origin. In the present case, the tumor cells did not stain positively for S-100 protein, despite the light microscopic suggestion of peripheral nerve origin. Ultrastructurally, most tumor cells contained a large number of myelinoid figures. This ultrastructural finding appears to be a useful diagnostic characteristics of neurothekeomas.