Objective Complex middle and inner ear malformations are considered an important limitation for cochlear implant (CI) with traditional microscopic techniques. The aim of the present study is to describe the results of the endoscopic-assisted CI procedure in children with malformed ears. Study Design Case series with chart review of consecutive patients. Setting Two tertiary referral centers: University Hospital of Verona and University Hospital of Modena, Italy. Subjects and Methods In total, 25 children underwent endoscopic-assisted cochlear implantation between January 2013 and January 2018. The audiologic and neuroradiologic assessment showed profound hearing loss and malformation of the middle and inner ear in all children. A complete review of anatomic features, surgical results, and audiologic outcomes was performed. The surgical technique is described step-by-step, and the outcomes are detailed. Results All patients (mean age, 3.6 years; range, 2.8-9 years) underwent a transattical/endoscopic-assisted CI procedure. All children showed varying degrees of auditory benefit, as measured by routine audiometry, speech perception tests, and Categories of Auditory Performance scores (mean, 6). No immediate or late postoperative complications were noted. Conclusion The endoscopic-assisted approach proved to be successful in cochlear implantation. The direct visualization and magnification allow (1) exploration of the tympanic cavity; (2) confirmation of all anatomic features, with strict control of the course of the facial nerve, round window area, and inner ear; and (3) performance of the cochleostomy with adequate insertion of the array.