The Authors report a case of a rare and very voluminous teratoma with uncommon location (the right cervico-facial region) and extracranial seat in a female newborn. The teratoma was excised in the 18th day of life. It showed cystic and solid lesions. The tissues were derived from the three germ layers and were prevalently mature; only a bit of them was represented by embryonic mesenchymal tissue. The baby, followed and checked up during all the first year of her life, showed a normal evolution and absence of recurrences.