Objective: To describe the etiology of advanced cases of external auditory canal (EAC) cholesteatoma (EACC), and to report the surgical management and outcomes based on canalplasty depending on the extent of adjacent structures involvement around the EAC. Study Design: Retrospective case review. Setting: University hospital. Patients: Participants comprised 28 patients (12 males, 16 females) ranging in age from 9 to 86 years old. All patients presented with advanced-stage EACC (Naim’s classification: stage III/IV). Intervention: Surgery ranged from simple bony meatoplasty with cartilage graft repair to more enlarged EAC canalplasty combined with tympanoplasty. Result: EACC was categorized as idiopathic in 22 patients and secondary EACC in six patients. Six patients with idiopathic EACC were receiving hemodialysis, one of whom showed bilateral pathology. Particularly in cases with epithelial invasion into the hypotympanum immediately adjacent to the jugular bulb, multi-layered repair including bone paste, post-auricular pedicled musculo-periosteal flap, auto cartilage, and temporalis muscle fascia were required. Five patients required revision surgery (17%), including one case of recurrent (reconstructed) cholesteatoma and three cases of perforation of the tympanic membrane. Conclusion: In the majority of our series, underlying diseases followed with microangiopathy and angiogenesis could be possible candidates in the etiology of EACC. Enlarged bony meatoplasty and multilayered reconstruction surgery brought about self-cleaning and dried the EAC in advanced-stage EACC.