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This clinical case report describes concomitant developmental disorders in the permanent dentition. The concurrence of bilateral dens evaginatus and dental fusion is a rare dental anomaly. Dental fusion is characterized by the union of 2 adjacent teeth at the crown level, which produces the formation of an enlarged clinical crown. Dens evaginatus is an enamel elevation similar to a cusp, generally located in the main groove of molars and premolars. The etiology of both anomalies is uncertain. The purpose of this paper was to review the related literature and present a clinical case where both anomalies are present.