Neurofibromas are benign peripheral nerve sheath tumors that can originate from several elements of peripheral nerves, including axons, Schwann cells, endoneurial fibroblasts, and perineurial cells. The occurrence of a solitary neurofibroma in the external nose, especially that isolated in the nasal columella, is extremely rare. To the best of our knowledge, only 4 cases of solitary neurofibromas in the external nose have been reported in the English literature: on the nasal dorsum, tip, and pyriform aperture, all originating from the trigeminal nerve. We report the first case of a solitary neurofibroma isolated in the nasal columella, which we found in an otherwise healthy 42-year-old man. We completely resected this tumor with a negative resection margin and performed reconstruction with a bilateral spreader graft and caudal septal extension graft using autologous septal cartilage. The postoperative course was successful in both cosmetic and functional results, with no sensory changes at the 1-year follow-up. Surgical treatment for this lesion was challenging due to the cosmetically obvious location and high rate of recurrence. A review of the literature highlights the clinical and histological characteristics, differential diagnosis, and management of solitary neurofibroma of the external nose.