Congenital cervical teratomas are benign but serious germinative cell tumors. The possibility of air compression structures and the risk of invasion of vascular and nervous noble elements are crucial prognostic factors in this kind of tumor. Prenatal diagnosis raises on ultrasound examination precising locoregional consequences of the tumor and surgical possibilities. In the propitious cases, prenatal MRI examination is useful to precise tumor’s limits and cerebral status of the foetus. At birth, coordinate management involving anaesthetists, paediatricians and specialized surgeons decrease morbidity and mortality. The recent observation of a case gave us the opportunity to make a literature review of this exceptional afection. It was a newborn female, aged 25 days allowed for anterior cervical mass evolving since the birth associated with moderate dyspnea. Cervical ultrasound coupled to CT scan had objectified a heterogeneous mass with cystic areas and calcifications. The excision was complete and the histopathological study revealed a mature teratoma. The postoperative course was uneventful, with some food and phonatory transient disorders. Cosmetic outcome at 6 months was excellent. Cervical teratoma is a rare tumor, diagnosed mainly in the neonatal period, which requires a prenatal diagnosis and multidisciplinary early management. The prognosis depends mainly on the presence of neonatal respiratory distress and histological form. There are opportunities sudden worsening respiratory and malignant transformation that justify early surgical excision and prolonged follow-up.