Abstract
Background: Teratoma is a tumor usually consisting of tissues derived from multiple germ layers. A congenital teratoma occurring in the region of the head and neck is rare with barely 10 reports in the global literature since 1996. Of further rarity is its mandibular location. This probably represents the first report of its kind in the world in addition to being the first one to be reported from Nepal. Case Presentation: We report a case of an 11-month-old infant with a congenital appendage over the side of the chin that was treated with surgical excision and histologically confirmed as mature teratoma. Conclusion: Congenital facial teratoma in the mandibular location is a rare event. A principle combining appropriate surgical technique and correct oncological principles keeping esthetic appearance in mind is necessary for the best outcome.