Enamel-renal syndrome is a rare autosomal recessive condition.

The dental abnormalities include This condition is caused by mutations in the FAM20A gene.

This gene encodes a protein – the Golgi apparatus associated secretory pathway pseudokinase.

This condition is usually diagnosed in childhood but may not be recognised until early adulthood.

[citation needed] There is no specific treatment for this condition currently known and management of its various features is the norm.