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Gastrointestinal involvement is noted in up to 25% of patients with neurofibromatosis. Most neurofibromas of the gastrointestinal tract are noted in the stomach and duodenum, the colon can be involved rarely. Most of these occur as a part of colonic involvement in NF1. Solitary neurofibroma of the colon is extremely rare. We present the case of a 13-year-old female having an isolated rectal neurofibroma without any systemic signs and family history of neurofibromatosis; very few numbers of such cases have been reported. The clinical significance of detecting these cases lies in the need of follow-up of these patients as it can be associated with or it can be the initial presentation of neurofibromatosis and there lies a risk of malignant transformation.