Congenital teratomas are considered to be neoplasms of maldevelopment and contain elements derived from more than one of the embryonic layers. The most common locations for teratomas in childhood include the sacrococcygeal region, gonads and mediastinum. Teratomas of the head and neck region are distinctly uncommon accounting for less than 5% of reported teratomas, but potentially lethal. Prompt diagnosis and early surgical excision are mandatory. In this report, we described a case of teratoma of the neck in a child treated by surgery.