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Ectodermal Dysplasia (ED) is a rare congenital syndrome that exhibits defects in two or more ectodermal tissues. Dental manifestations include delayed eruption of permanent dentition, complete absence of teeth or absence of a few teeth, and peg-shaped teeth. This condition is typically diagnosed by a dentist when parents bring their child with complaints of absent/delayed eruption of primary teeth. Hypohidrotic ED with true anodontia is an extremely rare condition, particularly in females. Over the past 50 years, only 11 cases have been reported involving anodontia of primary and permanent dentition in ED. The current report describes a case of a three-year-old female child diagnosed with hypohidrotic ED with anodontia, successfully treated by fabricating complete dentures with proper retention to restore function and aesthetics.