Allgrove syndrome (AS), also known as triple A syndrome, is an autosomal recessive inherited disorder characterized by a triad of alacrimia, achalasia, and adrenocorticotrophic hormone (ACTH) resistant adrenal insufficiency. We present a case of a 16-year-old male presenting with the classic triad and a homozygous mutation in the Aladin WD Repeat Nucleoporin (AAAS gene). Genetic analysis and detection of AAAS gene mutation is the cornerstone of diagnosis. Delayed detection results in multiple hospital admissions and life-threatening complications. This evidence concerns the gene AAAS and triple-A syndrome.