The triple A or Allgrove's syndrome is an autosomal recessive disorder 
characterized by the triad of achalasia cardia, alacrima and ACTH resistant 
adrenocortical insufficiency. Mutations of the 
Achalasia-Addisonianism-Alacrima-Syndrome (AAAS) gene on chromosome 12q13 are 
associated with this syndrome. We report an Indian family where two siblings 
were homozygous for a known mutation of the AAAS gene and presented with the 
classical triad of symptoms. The mother and the brother were heterozygous and 
asymptomatic. The affected siblings had iron deficiency anemia and the younger 
sister had pes cavus and palmoplantar keratosis. Neurological symptoms were 
absent in both affected children. Recognition of this syndrome can lead to early 
treatment of adrenal insufficency and genetic counselling.