We report a rare case of solitary neurofibroma on the eyelid margin without neurofibromatosis.

A 46-year-old male presented with a well-define small nodular lesion on the right upper eyelid margin that had not changed for 10 years. Surgical excision and biopsy were performed. Histological examination showed spindle-shaped cells in the fibrous stroma on hematoxylin & eosin staining, and immunohistochemical staining revealed S-100 protein-positive cells. Dermatologic, neurologic, and genetic evaluations showed no evidence of systemic neurofibromatosis. Six months after operation, there was no evidence of local recurrence.

To the best of our knowledge, this is the first case of solitary neurofibroma involving the eyelid margin without neurofibromatosis in Korea. Neurofibroma should be considered in a differential diagnosis of eyelid mass and can be successfully managed with surgical excision.