Facial nerve sheath tumors are the most common middle ear benign neoplasms. Four facial nerve sheath tumors in the middle ear were diagnosed in our department over a 12-year period, and we described our experience in the management of these tumors. A thorough review of the English literature on middle ear peripheral nerve sheath tumors has been conducted, and the findings of this review are presented and discussed. We have paid particular attention to the presenting features of these tumors with the aim of enhancing diagnostic ability.