True generalized macrodontia in a case of Rabson–Mendenhall syndrome

True generalized macrodontia in young children is an extremely rare occurrence reported in patients afflicted by a very uncommon disorder called Rabson–Mendenhall syndrome. This syndrome is an autosomal recessive disorder characterized by insulin resistance. A case with all the classical features of this syndrome i.e. insulin-resistant diabetes, acanthosis nigricans, hypertrichosis, deficiency of subcutaneous fat, growth retardation, coarse senile facies, large ears, macroglossia, furrowed tongue and dental prematurity along with marked generalized macrodontia of grotesque proportions in a 10-year-old girl is reported here, and the literature available is briefly reviewed.