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Taurodontism can be defined as a change in tooth shape caused by the failure of Hertwig′s epithelial sheath diaphragm to invaginate at the proper horizontal level. An enlarged pulp chamber, apical displacement of the pulpal floor, and no constriction at the level of the cemento-enamel junction are the characteristic features. Although permanent molar teeth are most commonly affected, this change can also be seen in both the permanent and deciduous dentition, unilaterally or bilaterally, and in any combination of teeth or quadrants. Whilst it appears most frequently as an isolated anomaly, its association with several syndromes and abnormalities has also been reported. Despite the clinical challenges, taurodontism has received little attention from clinicians. Due to the prevalence of taurodontism in modern dentitions and the critical need for its true diagnosis and management, this review addresses the etiology, clinical and radiographic features of taurodontism, its association with various syndromes and anomalies, as well as important considerations in various areas of expertise dental treatments of such teeth.