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Neurofibromas may occur as part of neurofibromatosis or as a solitary tumor. Solitary subungual neurofibroma appears to be a rare condition. Up until now, less than 10 case reports of solitary subungual neurofibroma have been documented. Solitary subungual neurofibroma is difficult to diagnose, particularly as it is often small and without obvious symptoms. Awareness of the diagnosis is emphasized to prevent unnecessary delay in treatment. So, understanding the differentiation and diagnosis of solitary subungual neurofibroma has a great value.A 32-year-old woman presented with a two-year history of painless thickening and elevation of the nail plate of the right first finger. She was treated with complete surgical excision of the tumor, and we performed pathological examination of the biopsy specimen.The final diagnosis of the tumor was solitary neurofibroma. After surgery, the nail regrew with a good cosmetic result. After 10 months of follow-up, there were no signs of recurrence of the tumor.Solitary subungual neurofibroma appears to be a rare condition, and immunohistochemistry is the key to the diagnosis. Complete surgical excision should be considered as the curative treatment of choice for them.