AAAS encodes aladin, a WD repeat nucleoporin essential for nucleocytoplasmic transport and mitotic spindle assembly. Aladin localizes to the nuclear pore complex and regulates the proper positioning of aurora kinase AURKA and the microtubule-binding protein NUMA1, ensuring correct spindle formation and timely chromosome 12 during mitosis 1. The protein also plays critical roles in peripheral and central nervous system development 234. Mutations in AAAS cause Allgrove syndrome (Triple-A syndrome), a rare autosomal recessive disorder characterized by the classic triad of ACTH-resistant adrenal insufficiency, alacrimia (dry eyes), and achalasia (esophageal dysfunction) 56. Most mutations produce truncated ALADIN proteins, though missense and point mutations have been reported 5. Neurological manifestations, including polyneuropathy, amyotrophy, and optic atrophy, typically emerge later in disease progression as autonomic complications 57. Clinical management involves glucocorticoid and mineralocorticoid replacement for adrenal insufficiency, artificial tears for alacrimia, and pneumatic dilatation or surgical myotomy for achalasia 56. Early recognition is important as alacrimia may be the earliest disease feature, though achalasia and adrenal insufficiency are more common presenting symptoms.