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.