Heterotopic brain is a rare entity. Histologically, this lesion resembles mature brain and often contains specialized tissues similar to choroid plexus or glia. Specialized neural stains are necessary to differentiate this rare anomaly from other tumors or conditions found in the head and neck. The differential diagnosis includes squamous cell carcinoma, granular cell tumor, hemangioma, lymphangioma, thyroglossal duct cyst, dermoid cyst, hamartoma, rhabdomyosarcoma, and teratoma. We describe a newborn with heterotopic brain tissue occurring on the dorsum of the tongue. We found only one other description of this developmental aberration in the English literature. Our patient was successfully treated with carbon dioxide laser excision of the mass. There has been no evidence of complication or recurrence after one year of follow-up.