Congenital cervical teratoma is a rare congenital malformation with an estimated incidence of one in 20,000-40,000 live births. Due to its mass effect it has a potential to cause significant fetal airway obstruction, while its hyper-vascularity may result in fetal heart failure and hydrops fetalis. An association with intracranial abnormalities has been previously described but is extremely rare and no cases of postnatal survival have previously been reported. In this study we report the first case of congenital cervical teratoma with neuronal migration disorder in a live-born infant and the role of in-utero cyst drainage as an airway salvage intervention. We also present a literature review on etiology, diagnosis, management options and prognosis of congenital cervical teratoma with and without intracranial abnormalities.