Ankyloglossia or tongue tie is a congenital anomaly with a prevalence of 4-5% and characterized by abnormally short lingual frenulum. For unknown reasons the abnormality is 2 times more common in males. The pathogenesis of Ankyloglossia is not known. The authors report a family with isolated Ankyloglossia inherited as an autosomal dominant trait in four consecutive generations. The identification of the defective gene (s) in these patients might reveal novel information on the pathogenesis of this disorder. This case report elucidates a specific inheritance pattern of tongue tie.