This article presents a clinical case of keloid scar removal after canaloplasty for congenital atresia of the external auditory canal. The interest of this clinical case lies in the rare occurrence of a keloid scar of the external auditory canal, leading to its total obturation. Scarring processes in the external auditory canal are an important problem that significantly affects the hearing of patients. Acquired fibrous atresia is represented by a conglomerate of soft tissues in the bony part of the external auditory canal, soldered to the outer surface of the eardrum. Keloid is an excessive growth of scar tissue in the dermis and underlying tissues with hyalinosis of bundles of collagen fibers. We described the surgical treatment of a keloid scar that appeared 11 months after the canaloplasty for congenital atresia of the external auditory canal. Previously, the patient underwent two local injections with an interval of 1 month into the anterior and posterior sections of the keloid scar of the prolonged glucocorticosteroid Triamcinolone in order to reduce the size of the pathological tissue. After removal of the keloid scar, totally obstructing the external auditory canal, the patient noted an improvement in hearing with a decrease in bone-air gap by 25 dB compared with the preoperative study.