Neurothekeoma is a benign dermal soft tissue tumor that is probably of nerve sheath origin. It usually presents as a slow-growing, solitary papule or nodule that is mainly located on the head and neck or upper extremities except for the hand. There are three histologic subtypes-myxoid, mixed and cellular type-depending on the amount of myxoid stroma. Immunohistochemical markers such as S-100, glial fibrillary acidic protein, nerve growth factor receptor, NK1C3 (CD57) and Ki-M1p can be applied to the tumor cells to distinguish among thesethree subtypes of neurothekeoma. Only one case has been previously reported on the hand in Korea. Herein, we report a rare case of myxoid neurothekeoma on the hand with a review of the related literature. (Korean J Dermatol 2009;47(8):982~985)