Introduction: Type 1 Neurofibromatosis (NF1) is a rare pathology with heterogeneous clinical presentation and only a few cases involving the external auditory canal (EAC) have been reported so far (1-3). Objective: To report a case of NF1 with EAC involvement and literature review Discussion and Conclusions: Size and location of tumors in NF1 most influence presentation of clinical symptoms. Complete tumor resection is possible only in patients with small tumors. Patients with single neurofibromas of the head and neck can benefit from surgery in order to 1) exclude malignancy in a rapidly enlarging mass; 2) enhance cosmesis in those with disfiguring disease; 3) provide relief from neurogenic pain; 4) improve symptoms caused by compression.