Nerve sheath myxoma, a rare benign dermal soft tissue tumor of the peripheral nervous system, often occurs on the head, neck and upper extremity but rarely found in the lower lip. The case of nerve sheath myxoma in lower lip has not been reported in korea. Recently, we experienced an unusual case of nerve sheath myxoma involving the lower lip in a 34-year-old woman, reported a case of nerve sheath myxoma in lower lip with a review of the literature.