Amelogenesis imperfecta (AI) is a congenital disorder which presents with a rare abnormal formation of the enamel[1] or external layer of the crown of teeth, unrelated to any systemic or generalized conditions.

Enamel hypoplasia manifests in a variety of ways depending on the type of AI an individual has (see below), with pitting and plane-form defects common.

[4] The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes as well as rapid attrition, excessive calculus deposition, and gingival hyperplasia.

[citation needed] Amelogenesis imperfecta is also inherited in an autosomal recessive pattern; this form of the disorder can result from mutations in the ENAM, MMP20, KLK4, FAM20A, C4orf26 or SLC24A4 genes.

AI can be classified according to their clinical appearances:[19] Differential diagnosis would include dental fluorosis, molar-incisor hypomineralization, chronological disorders of tooth development.

[22] Preventive and restorative dental care is very important as well as considerations for esthetic issues since the crown are yellow from exposure of dentin due to enamel loss.

[20] Many factors are to be considered to decide on treatment options such as the classification and severity of AI, the patient's social history, clinical findings etc.

[citation needed] Full-coverage crowns are sometimes being used to compensate for the abraded enamel in adults, tackling the sensitivity the patient experiences.