Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc. Objective: To evaluate the outcome and per-and postoperative complications of the surgical management of patients with a persistent stapedial artery (PSA). Methods: A systemic literature search for reports on patients treated for pulsatile tinnitus and/or conductive hearing loss caused by a PSA was conducted of the PubMed and Embase databases using the terms "stapedial" and "artery." Inclusion criteria were adequate description of the intervention and pre-and postoperative signs and symptoms. In addition, one case of a PSA, treated at VU University Medical Center Amsterdam, The Netherlands, was included in this series. Intervention: Middle ear surgery consisting of stapedotomy or stapedectomy, and/or transection of the PSA. Main Outcome Measures: Pre-and postoperative hearing levels, pre-and postoperative pulsatile tinnitus, and per-and postoperative complications. Results: Seventeen patients and 18 operated ears were evaluated (16 patients described in 14 articles and our case). Twelve out of 14 ears in which a stapedotomy or stapedectomy was initiated experienced improvement in hearing. In four cases pulsatile tinnitus was described pre-and postoperatively. In all four, pulsatile tinnitus subsided after transection of the PSA. Peroperative bleeding from the PSA was described in four patients, which could be controlled during the procedure. No significant postoperative sequelae were reported. Conclusions: In case of a PSA, improvement of conductive hearing loss is best achieved by stapes surgery, while pulsatile tinnitus is effectively treated with transection of the PSA. To date no long-term postoperative complications have been reported.