Teratomas are tumors of germ cell derivation consist of tissues derived from all the three germ cell layers. They comprise the most common extragonadal germ cell tumors (EGCT) in childhood. EGCTs of head and neck region account for only 5% of all benign and malignant germ cell tumors and for 6% of all teratomas. Primitive neuroectodermal tumor (PNET) arising from germ cell tumor is a distinct entity. It develops from the malignant transformation of teratomas along ectodermal lines. This paper presents a rare case of oral teratoma in a 6-year-old male who reported with pain and swelling in the right upper back teeth region of jaw. Under light microscopy, mature and immature structures representatives of trilineage derivatives were appreciated. Sheets of small round cells showing vague rosette and membranous positivity to CD-99 directed us to consider the above diagnosis.
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