Abstract
Triple A syndrome, characterized by the triad of alacrimia, achalasia, and adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency, is a rare and often underreported condition. We present the case of a 3-year-old boy who presented with hypoglycemic seizures unresponsive to glucose boluses. A detailed history revealed congenital alacrimia. The suspicion of Triple A syndrome led to the initiation of hydrocortisone therapy, which resulted in rapid symptom resolution and confirmed the diagnosis. Early recognition of this multisystemic genetic disorder is crucial, as a delay in diagnosis can be life-threatening.