Ankyloglossia is a congenital condition characterized by a short lingual frenulum, which may result in the restriction of tongue movement and function. Considerable controversy regarding the diagnosis, clinical significance, and management of the condition remains, and great variations in practice have been recorded. Indeed, attitudes toward ankyloglossia differ among professional groups, and opinions may vary remarkably even among those within the same specialty. This article reviews the embryology, genetics, diagnosis, clinical presentation, and treatment of ankyloglossia to help physicians better understand and treat the condition.