INTRODUCTION: Allgrove syndrome (AS) is a rare autosomal recessive disorder 
characterized by achalasia cardia, alacrimia, and adrenocorticotropic 
hormone-resistant adrenal insufficiency which is sometimes associated with 
autonomic dysfunction. It has also been referred to as the triple A syndrome in 
view of the cardinal symptoms described above. First described by Allgrove et al 
in 1978, the disorder usually presents mostly during the first decade of life. 
These patients have the threat of adrenal crisis, shock, and hypoglycemia and 
are usually on steroid supplementation.
CASE REPORT: The anesthesiologist's encounter with such patients, although rare, 
is mostly for repair of the achalasia cardia. We thus report a similar case of 
AS in a 2-year-old girl who was scheduled to undergo Heller myotomy along with 
the preoperative evaluation and intraoperative management of the same.
CONCLUSION: Being aware of the pathophysiology of AS gives useful insight about 
the disease and successful perioperative management in the form of the triple S 
strategy, that is, stress dose of steroids, slow induction and positioning, and 
finally maintenance of stable hemodynamics and euglycemia.