PURPOSE: Neurofibromas may present as multiple or solitary lesions. Although there is no predilection site for solitary lesions, they are rare on the hand. In addition, solitary intramuscular neurofibromas are a very rare pathological type. Here, we report a rare solitary intramuscular neurofibroma in the hand. This paper examines the clinical characteristics of intramuscular neurofibroma arising from the lumbricalis in order to enable a correct diagnosis and treatment. METHODS: A 32-year-old male presented with a painless mass on the palm. The physical examination revealed a 3 x 2cm protruding mass that was non-tender to palpation. The vascular and sensory examinations were unremarkable, while the motor examination showed mild difficulty with flexion and extension. Magnetic resonance imaging demonstrated an enhancing solid mass between the thenar eminence and second metacarpophalangeal joint. The diagnosis of an intramuscular neurofibroma was confirmed following surgical excision and histological evaluation. RESULTS: The pathological examination was consistent with a neurofibroma, with delicate fascicles and loose fusiform cells in a fibrous stroma, with oval or spindle-shaped nuclei and scant cytoplasm. The background matrix was pale staining and had focal myxoid stroma. There was no significant nuclear pleomorphism and no mitoses. Immunohistochemistry with S-100 was slightly positive. At the 6-month follow-up, motor and sensory function were intact and the range of motion was full. CONCLUSION: A neurofibroma is a rare tumor of the hand, especially the intramuscular type. Hand surgeons should consider the diagnosis of this tumor based on the examination and imaging.