Background and Aim: The Donohue syndrome, also known as leprechaunism, is a rare autosomal recessive genetic disorder of insulin resistance. The severe form of insulin resistance seen in this syndrome is due to a mutation in the insulin receptor gene. This syndrome has symptoms such as delayed growth before and after birth, premature growth, lipoatrophy, hypertrichosis, acanthosis nigricans, and dysmorphic face.
Case Presentation: This report presents oral manifestations (such as macrodontia, severe crowding, supernumerary tooth, etc.) and management of a 10-year-old patient with the Donohue syndrome.
Conclusion: Considering the extensive oral manifestations of the Donohue syndrome, good oral hygiene and regular dental follow-ups are necessary for such patients.