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Rare dental anomalies can present in both syndromic and non-syndromic patients. They can be cosmetically objectionable and may require complex dental interventions. Anomalies such as lobodontia, globodontia, and solitary maxillary central incisors occur in specific syndromes, while others like facial talon cusp and pyramidal molars can be isolated findings.

Anomalies of tooth eruption may occur in one tooth, multiple or all teeth of a dentition. Disruption during the eruption stage of tooth development, genetics, or environmental disturbance may affect eruption of primary incisors, primary molars, first permanent molars, maxillary permanent canines and interchange of the position of teeth. Treatment options include restorative, prosthetic, orthodontic or surgical intervention with the aim of achieving acceptable function and orthodontic development.

Anomalies of crown shape often occur in the permanent dentition. Heredity, chromosomal abnormality, environmental forces, traumatic injury, and mutations result in various anomalies of crown shape. As with crown size, the shape of teeth is created during morphodifferentiation in the Bell stage of tooth development.

Variations in tooth number represent one of the more common dental anomalies. Tooth agenesis, or failure of odontogenesis, may be total or partial, resulting in the absence or decreased number of teeth. Hyperdontia, or increased number of teeth, is a disorder of proliferation during odontogenesis. Genetic influences and multiple syndromes are associated with both anomalies.

Anomalies of tooth formation include diverse entities that vary in severity. Some of the anomalies can only be diagnosed with radiographic examination while others are clinically evident. Early diagnosis of some of these conditions is crucial in order to avoid further complications in patients such as pulp necrosis and abscess formation. Of these anomalies some present more frequently in the primary dentition and can have consequences in the developing permanent teeth.

Dentin is located within the crown and root of both primary and permanent teeth. Anomalies that affect the formation of dentin can result in abnormal tooth wear, abnormal tooth form, and can result in subsequent breakdown of the dentition with associated symptoms. The etiology is genetic with resultant varying phenotypic presentations based on the type of gene defect.

Anomalies of crown size may occur in one tooth, multiple teeth, or all teeth within the dentition. Disruption during the physiologic process of morphodifferentiation, genetics, environmental disturbance, chemotherapy, irradiation, and multiple syndromes is associated with crown size. Treatment options include restorative, prosthodontic, orthodontic, and/or surgical intervention with the aim of achieving acceptable function and esthetics.

Anomalies that affect the formation of enamel are due to genetic, systemic, local, or unknown causes. In general, defects in enamel formation due to either systemic or local etiologies will have various presentations depending upon which stage of enamel formation the insult occurred. In addition, the mode of inheritance (the molecular basis) will result in various visual presentations of enamel defects.