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Enamel hypoplasia secondary to amelogenesis imperfect (AI) is one of the common developmental disturbances associated with the oral cavity. AI in association with multiple unerupted teeth is a rare entity, and in adolescence it not only has an affect on esthetics but also has an impact on the psychological status of the person. AI has been reported with other systemic anomalies previously. We report a case of AI in association with multiple unerupted teeth and nephrocalcinosis in siblings. The present case also highlights the importance of systemic examination and investigations in planning the treatment of a patient with AI. 

Amelogenesis Imperfecta with Nephrocalcinosis: A Rare Association in Siblings