Abstract:
Amelogenesis imperfecta (AI) is a group of hereditary disorders that affect the quality and/or quantity of dental enamel. AI also
named enamel-renal syndrome, is an extremely rare syndrome which is characterized by hypoplastic amelogenesis imperfecta
(hypoplastic dental enamel) and nephrocalcinosis (precipitation of calcium salts in renal tissue). Patient presented oral
clinicopathological manifestations, includes permanent dentition, alterations in the tooth shape, reduction of the enamel thickness
and yellow discoloration with many teeth pulpal exposed, delayed tooth eruption, and intrapulpal calcifications. Nephrocalcinosis
is often asymptomatic but can progress during late childhood or early adulthood to impaired renal function.
