Several neoplastic lesions may originate from facial nerves, including facial nerve schwannomas. These neoplastic lesions can cause various symptoms, such as facial nerve paralysis, and decrease the quality of life of patients. Therefore, knowledge of how to manage these diseases is important for otologists. However, the incidence of these diseases is extremely low, and universal management methods have not yet been established. This review summarizes recent advances in knowledge regarding these neoplastic lesions, especially facial nerve schwannomas.Recent advances and the accumulation of knowledge regarding these benign facial nerve lesions have provided several preferable treatments and management methods, especially for facial nerve schwannomas. However, this still depends on the patient's symptoms and tumor localization.This review presents the optimal treatment protocol and differential diagnosis of benign facial nerve lesions. This may be useful for pretreatment differentiation and treatment decision-making.