Background External auditory canal (EAC) cholesteatoma is a lesion lined with stratified squamous epithelium containing proliferative keratin with bony erosion in EAC which can spread to the tympanic cavity, mastoid, and surrounding organ. External cholesteatoma can occur in patients with congenital abnormalities such as congenital aural atresia (CAA). Method This case series was reported using the 2020 PROCESS Guideline. The design of this study used a retrospective study during the 2015–2020 period. Result 3 participants aged 10.67 ± 2.31 years with CAA had other complaints of ear infections. All participants experienced sensorineural hearing loss with an average threshold of 59.33 ± 36.68 dB and suspicious cholesteatoma from a CT scan. Canal wall down, meatoplasty, and/or canaloplasty were performed based on the findings. Conclusion Surgical procedure in CAA with cholesteatoma aimed on preventing further complications and recurrence.