Autoimmune polyglandular syndrome type 1 exhibits very specific oral manifestations in the form of enamel hypoplasia and oral candidiasis. The authors present the case of a 10-year-old girl with autoimmune polyglandular syndrome characterized by chronic mucocutaneous candidiasis infections, hypoparathyroidism, and enamel hypoplasia. The importance of this entity is stressed, with special attention to the observed oral anomalies.