Nerve sheath myxoma or neurothekeoma, a rare cutaneous neoplasm probably originating from the nerve sheath, is classified into three groups as classical, cellular, and mixed types based on cellularity, mucin content and growth patterns. Only two cases of nerve sheath myxoma have been reported in the Korean literatures. We report a case of nerve sheath myxoma occurring on the scalp of a 29 year-old woman. It was identified as the mixed type by histopathological, immunohistochemical and electronmicroscopic findings. (Korean J Dermatol 1998;36(2): 367-371)