Association of enamel-renal syndrome with sialolith: A rare entity

Amelogenesis imperfecta (AI) is a disease primarily affecting amelogenesis, but other various aberrations have been reported in association with this entity. Enamel-renal syndrome (ERS) is a very rare disorder associating AI with nephrocalcinosis. It is known by various synonyms such as AI nephrocalcinosis syndrome, MacGibbon syndrome, Lubinsky syndrome and Lubinsky–MacGibbon syndrome. The purposes of this article are to describe other dental anomalies which are rarely associated with AI making the diagnosis of AI a complex disorder and also to report a rare case of MacGibbon syndrome presented with sialolith of submandibular gland duct which is the first reported case in the literature.