Teratomas, most often diagnosed in younger patients, represent the most frequently identified subtype of pediatric germ cell tumors. It is very uncommon for teratomas to present in the head and neck region and demonstrate malignant transformation. We present a case of squamous cell carcinoma arising in an alpha‐fetoprotein‐producing cystic teratoma of the mandible in a 2‐year‐old female that is, to the best of our knowledge, the first such published report. The patient was treated with surgical excision along with chemotherapy and has remained disease‐free 2 years after the conclusion of therapy. Pediatr Blood Cancer 2009;52:130–132. © 2008 Wiley‐Liss, Inc.