The most common oral choristomas are consisted of thyroid tissue and bone. The presence of sebaceous glands in the oral mucosa, especially in the buccal mucosa and labial mucosa, is often considered a normal anatomical variation since they are observed in about 80% of the population and are called ectopic sebaceous glands or Fordyce's granules. However, the presence of these glands on the tongue is rare, with only 11 cases in the dorsum of the tongue reported in the English literature, and it is considered a choristoma. This paper aims to report the third case in the literature of a congenital sebaceous choristoma on the tongue. An 11-year-old white male patient presented a firm sessile papule, without color alteration, measuring 0.4 cm x 0.3 cm in diameter, in the middle third of the dorsum of the tongue with a slight increased size in the last months. Histopathological examination showed an invagination of the epithelium into the connective tissue, forming a ductal structure covered by stratified squamous epithelium. The deeper areas had normal well-differentiated sebaceous glands, with ducts connected to the central duct. Considering clinical and histopathological findings the diagnosis was sebaceous choristoma. Despite being rare, sebaceous choristomas should also be considered in the differential diagnosis of tongue abnormalities or lesions. Although the pathogenesis is not well understood, the present report, as a congenital choristoma case in the midline, reinforces the hypothesis of a disorder with embryological origin and a possible relationship with thyroglossal duct remnants. Key words:Choristoma, Oral Mucosa, Tongue.
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