BACKGROUND Allgrove syndrome, or triple "A" syndrome (3A syndrome), is a rare 
autosomal recessive syndrome with variable phenotype, and an estimated 
prevalence of 1 per 1,000,000 individuals. Patients usually display the triad of 
achalasia, alacrima, and adrenocorticotropin (ACTH) insensitive adrenal 
insufficiency, though the presentation is inconsistent. CASE REPORT Here, the 
authors report a case of Allgrove syndrome in a pediatric patient with delayed 
diagnosis in order to raise awareness of this potentially fatal disease as a 
differential diagnosis of alacrima. CONCLUSIONS The prevalence of Allgrove 
syndrome may be much higher as a result of underdiagnosis and missed diagnosis 
due to the variable presentation and sudden unexplained childhood death from 
adrenal crisis. The authors review the characteristic symptoms of Allgrove 
syndrome in relation to the case study in order to avoid missed or delayed 
diagnosis, potentially decreasing morbidity, and mortality in those affected by 
this disease.