RESUMO
BACKGROUND: We report a rare case of first branchial cleft anomaly (FBCA) accompanied by bony atresia of the external auditory canal, middle ear malformation, and location malformation of the facial nerve according to the intraoperative findings. CASE SUMMARY: A 19-year-old male patient presented to our department with a mass behind the right earlobe and recurrent postauricular swelling and pain since childhood, he also had severe hearing loss in the right ear since birth. The patient underwent surgery including mass removal, mastoidectomy, and simultaneous meatoplasty and ossiculoplasty under microscopy. No facial palsy or recurrence was noted during postoperative follow-up. CONCLUSION: FBCAs are rare, and to our knowledge, this is the first report of FBCA accompanied by external auditory canal bony atresia, middle ear malformation, and location malformation of the facial nerve. An effective postauricular approach under microscopy facilitated complete lesion removal and simultaneous otologic reconstruction.