A 7-year-old boy whose features included microtia, external-ear canal stenosis, congenital cholesteatoma in the middle ear and mastoid with postauricular abscess and automastoidectomy and a first branchial cleft fistula opening in the middle ear through the zygomatic root was referred to our clinic (). Discharge from the periauricular sinus with wide tract had been occurring since 3−4 years previously. Discharge ended upon antibiotic treatment, but was reported again after cessation of treatment. In a clinical examination, we observed severe auditory canal stenosis and 55-db conductive hearing loss in pure tone audiogram. These results were consistent with tuning tests. Facial nerve function was normal. Imaging studies such as computed tomography (CT) showed the extent of the bony erosion in the mastoid air cells (automastoidectomy) with cortical fistula to the skin. The middle ear and mastoid were filled with the appearance of soft tissue (). The first branchial fistula and its tract to the zygomatic root were shown by fistulography (). An exploration of the mastoid through a postauricular approach, with the fistula site in the incision, was planned. One large mastoid cavity was found to be full of cholesteatoma, extending from the anterior wall of the middle ear where the branchial fistula was opening and completely obliterating the external auditory canal, middle ear and mastoid cells with ossicular chain erosion. Canal wall down mastoidectomy with canaloplasty and wide meatoplasty was performed. The Eustachian tube was also cleaned completely. The branchial cleft fistula from the anterior auricle to the zygomatic root was excised through parotidectomy and facial nerve dissection (). The tract of the first branchial fistula crossed the superior branch of the facial nerve. There was a hole in the zygomatic root in which the epithelium was extended to the middle ear. The tract was excised completely and confirmed pathologically as squamous epithelium. The site of the operation was closed. Facial nerve function was normal in the post-operative period. No evidence of recurrence was observed during a 6-month follow-up period.