Gastrointestinal involvement in Neurofibromatosis type 1 (NF1) is uncommon and colonic neurofibromas are rarely reported. We report an interesting case of a colonic neurofibroma in a patient with NF1, highlighting its clinicopathological features and differential diagnoses. The lesion manifested as a mucosal polyp in the sigmoid colon and on histopathologic examination showed fascicles of spindle cells with wavy nucleus and S100 positivity, confirming a neurofibroma. Recognition of this uncommon manifestation is important to distinguish it from other mesenchymal lesions of colon and guide appropriate clinical management and follow -up