To study congenitally deaf children with inner ear malformations that usually have comorbid anomalous facial nerves and middle ear deformities. To determine the feasibility of endoscopy-assisted transmeatal cochlear implantation with the purpose of reducing the risks of iatrogenic facial nerve injury. This report presents a unique technique in a pediatric case with multiple ear anomalies: microtia, cochlear hypoplasia with an aberrant facial nerve, a sigmoid sinus deformity leading to a narrow mastoid cavity, and a flat promontory wall without round window. A cochlear implant electrode array was successfully inserted endoscopically using the transmeatal approach in the present case. It caused no postoperative surgical complications, and the patient was then able to hear binaurally and functionally. For patients with comorbid multiple ear deformities, using endoscopy for cochlear implantation transmeatally is an alternative method providing a better visualization of the middle ear anatomy, an avoidance of injury of facial nerve, and an assurance of precise insertion of the electrode when the posterior tympanotomy approach is not applicable.