Ankyloglossia superior syndrome is a rare malformation that consists of a fibrous or osseous connection between the tip of the tongue and the hard palate, and additional congenital anomalies such as cleft palate, gastrointestinal malformations, and deformed limbs. We present the case of a 5-year-old boy with ankyloglossia superior syndrome that comprised the complete complex malformation. We reviewed previous publications and summarise the different theories of its genesis.
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