Enteric duplication cysts (EDCs) are congenital anomalies. We report the case of a 5-day-old girl with a 2.0 cm congenital oral mass in the tongue, an extremely uncommon site of EDC. The tongue mass was resected without complication; microscopic findings were characteristic of an EDC, and the patient is doing very well. The English literature was researched for the cases of single congenital oral cavity masses diagnosed prenatally or at birth as EDC or EDC-like lesions. Cystic lesions of the oral cavity partially lined by gastrointestinal epithelium, without teratoma features, have received several names. Similarities between EDC and EDC-like lesions favor the idea of one lesion with several morphologies manifest along a spectrum and that the necessity for EDC diagnosis of the smooth muscle coat criteria could be re-evaluated to improve the categorization of these lesions and better understand the pathogenic mechanism.

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