Teratomas are rare congenital tumors. Teratomas frequently occur in adult ovaries, but can be located primarily in the sacrococcygeal zone and intracranial compartment in newborns. Teratomas are rare in the head and neck region. Teratomas arise following a sequence of cells derived from more than one germ layer at different regions of the body due to a change in location of germ cells, and contain ectodermic, endodermic, and mesodermic tissues. Herein we detail the follow-up and treatment of a newborn with a mass that was located at the back of the head and was referred to our Neurosurgery Clinic with a pathologic diagnosis of a grade II-III immature teratoma resembling an encephalocele.