Allgrove syndrome, also known as Triple A syndrome, is a rare autosomal 
recessive genetic disease characterized by three signs: esophageal achalasia, 
adrenocorticotropic hormone refractoriness, and alacrima. A 31-year-old male 
presented to our hospital for treatment of difficulty swallowing caused by 
esophageal achalasia. Because he had complicating alacrima, a neurologic 
disease, and a family history of consanguineous marriage, a genetic neurologic 
disease was suspected. Then, a mutation in the achalasia-addisonianism-alacrima 
syndrome gene was identified. With the diagnosis of Allgrove syndrome, a 
per-oral endoscopic myotomy (POEM) was performed for esophageal achalasia. After 
the POEM, the symptoms and the esophageal pressure findings ameliorated quickly, 
with no recurrence noted throughout a follow-up of more than 1 year. Here, we 
report the first case of POEM performed for esophageal achalasia in Allgrove 
syndrome.