Esophageal achalasia is characterized by abnormal peristaltic movements of the esophageal body and impaired relaxation of the lower esophageal sphincter (LES). However, its etiology remains unknown. In our previous study, it was shown that in the LES of patients with achalasia, hsv1-miR-H1 was overexpressed, ATG16L1 expression was downregulated and interleukin (IL)-1β levels were upregulated. However, systemic features were not evaluated. Herein, the plasma cytokine levels in patients with achalasia were determined. Plasma was collected from patients at Nagasaki University Hospital between February 2013 and March 2016, both before and after peroral endoscopic myotomy (POEM). Cytokine analysis was performed using plasma collected from 10 healthy individuals (control group) and 12 patients with achalasia using the Bio-Plex Pro™ Human Cytokine 27-plex assay kit. The levels of IL-17, IL-1β, C-C motif chemokine ligand 2, IL-4, IL-5, IL-1ra, IL-7, IL-12, interferon-γ, IL-2, fibroblast growth factor-2, colony-stimulating factor (CSF)2 and CSF3 were significantly higher in patients with achalasia compared with the control subjects. However, the levels did not differ between plasma samples collected before and after POEM. Thus, the occurrence of a cytokine storm was confirmed in the patients with achalasia.