Allgrove (Triple A) syndrome is a rare autosomal recessive disorder 
characterized by cardinal features of adrenal insufficiency due to 
adrenocorticotropic hormone (ACTH) resistance, achalasia, and alacrimia. It is 
frequently associated with neurological manifestations like polyneuropathy. 
Since its first description by Allgrove in 1978, approximately 100 cases have 
been reported in the literature. Here we report an 18-year-old boy diagnosed as 
having Allgrove syndrome, with ACTH resistant adrenal insufficiency, achalasia, 
alacrimia, and severe motor polyneuropathy. Alacrimia was the earliest feature 
evident at the age of 8 years. He presented with achalasia and adrenal 
insufficiency at 12 and 18 years respectively and developed neurological 
symptoms in the form of severe muscle wasting at the age of 15 years. Patients 
with Allgrove syndrome usually manifest adrenal insufficiency and achalasia 
during first decade of life. Our patient manifested adrenal insufficiency and 
achalasia in the second decade and manifested neurological dysfunction before 
adrenal dysfunction.