Allgrove syndrome which is known as the triple A syndrome, is a rare autosomal recessive disease. It has an estimated prevalence of 1 per 1,000,000 individuals. The triple A stands for the three most prominent features of the syndrome, alacrimia, achalasia and adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency. It may be associated with autonomic, central and peripheral nervous system abnormalities then the name 4 A syndrome has been introduced. In this paper, we report the case of a 14-year-old girl with the 4 A syndrome who was referred to the Oral Medicine Department of hospital La Rabta (Tunisia). She suffered from many oral manifestations of Allgrove's syndrome as well as general abnormalities. Through this observation, we will show that the Knowledge of the oral manifestations is important and it leads the dentist to the right prevention and management of bucco dental complications of these patients.