A teratoma is a true neoplasm composed of multiple tissues foreign to the sites from which they originate. The estimated incidence of mature congenital teratomas at all sites is 1 in 4000 live births, of which at least 2% are oropharyngeal. An epignathus tumor is a congenital malformation classified as a mature teratoma. The incidence of epignathus is much rarer, estimated from 1:35,000 to 1:200,000 live births and has a female predominance. Teratomas, by definition, are neoplasms that consist of all 3 germ cell layers, and in the case of epignathus teratomas, the germ cell layers are mature differentiated tissue. A 1-day-old infant presented to us with giant epignathus that arose from the palate and extended to the lateral oropharynx and to the tongue. The tumor did not cause immediate respiratory obstruction, but there was difficulty with feeding. At the fifth day of the baby's life, the tumor was excised completely under general anesthesia through an endotracheal intubation.

First branchial cleft anomaly is a rare disease of the head and neck. It accounts for less than 8% of all branchial abnormalities. It is classified into type I, which is thought to arise from the duplication of the membranous external ear canal and are composed of ectoderm only, and type II that have ectoderm and mesoderm. Because of its rarity, first branchial cleft anomaly is often misdiagnosed and results in inappropriate management. A 9-year-old girl presented to us with fistula in the submandibular region and discharge in the external ear. Under general anesthesia, complete surgical excision of the fistula tract was done through step-ladder approach, and the histopathologic examination confirmed the diagnosis of type II first branchial cleft anomaly.

Hypoglossal schwannomas usually develop in the intracranial portion of the brain. Incidence of hypoglossal schwannomas of the submandibular region is extremely rare. A 27-year-old patient presented to us with hypoglossal schwannoma of the tongue and the submandibular region. The tumor was excised intraorally combined with submandibular approach. Histopathologic examination revealed a plexiform schwannoma (a rare variant).