Persistent stapedial artery (PSA) is a rare congenital vascular anomaly, with both clinical and surgical implications. When encountered during middle ear surgery, it represents a challenge to the otologic surgeon, since damage to this artery could lead to massive intraoperative bleeding and poor surgical exposure. Here we report the case of a 3-year-old boy with prelingual and profound bilateral sensorineural hearing loss who was referred to our Otolaryngology-Head and Neck Surgery Department for cochlear implant evaluation. Pre-operative computed tomography (CT) examination of the temporal bone confirmed the diagnosis of PSA. Further evaluation showed no contraindication for surgery and the patient underwent cochlear implantation using the classic transmastoid, facial recess approach. During surgery the vessel was coursing far enough from the round window niche, making it possible to achieve safe and complete electrode insertion into the scala tympani of the cochlear basal turn. This case demonstrates that cochlear implantation is safe and feasible in patients with PSA. Thorough analysis of the preoperative temporal bone CT scan might help with surgical planning by identifying this anomaly prior to surgery.