Horn-shaped Symmetric Nodules on the Earlobes: An Unusual Presentation of First Branchial Cleft Anomaly

First branchial cleft anomaly (BCA) is a rare congenital malformation of the head and neck, <10% of all BCA. it occurs between the first and second branchial arches. The diagnosis of first BCA is often delayed because of its atypical shape and rarity. A 30-year-old male presented to our clinic with bilateral earlobes nodules, he first noticed 20 years prior. The lesions had gradually increased in size, and he stated no other specific symptoms. Bilaterally, a 1-cm-sized, sharp, horn-shaped, dark-colored, keratinized mass was palpated hardly, and no infectious signs were observed. Bilateral resection was performed, and the lesions did not form a tract in any other direction. Histopathology confirmed the lesions were first BCA composed of a cystic cavity lined by stratified squamous epithelium. Surgeons should suspect first BCA in patients with nonspecific lesions of the earlobe, and our case will help with future diagnoses.