Tabriz University of Medical Sciences Journal of Research in Clinical Medicine 2717-0616 13 1 2025 01 01 Ankyloglossia superior syndrome: a challenging case report and literature review 34481 34481 10.34172/jrcm.025.34481 EN Shahin Abdollahifakhim https://orcid.org/0000-0003-2141-2031 Faezeh Ghaderyan https://orcid.org/0009-0004-1556-7082 Hassan Soleimanpour https://orcid.org/0000-0002-1311-4096 Journal Article 10.34172/jrcm.025.34481 2023 05 30 2024 04 17 Ankyloglossia superior (palatoglossal adhesion) is an exceedingly uncommon congenital disorder, with just 20 cases recorded in the medical literature. This abnormality is considered to be a part of the ankyloglossia superior syndrome (ASS) when it occurs with other congenital abnormalities such as gastrointestinal anomalies, cleft palate, and limb deformities. We describe the uncommon occurrence of a cleft palate, limb deformity, and ASS with a synechial band extending from the incisive foramen to the tip of the tongue. In addition, surgical repair is detailed. Considering the origin of ASS, we reviewed the current research and explored several hypotheses. When a newborn is diagnosed with ankyloglossia superior, clinicians must have a high level of suspicion for additional congenital defects.