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Achalasia is a disease characterized by primary esophageal dysmotility, and its etiology remains unknown. Some population-based studies have suggested that achalasia patients have a higher risk of esophageal adenocarcinoma, but most studies have not evaluated the association between achalasia and esophageal squamous cell carcinoma (ESCC). The aims of the study were to examine the possible association between achalasia and ESCC in a national cohort and to compare the rates of ESCC development among achalasia patients and controls. This study was based on a large-scale national cohort consisting of more than 3.7 million patients. All the patients with primary achalasia diagnosis (ICD-10: ICD-10:M3239) or secondary achalasia diagnosis (ICD-10: ICD-10:M3385) identified between 1998 and 2006 from the Taiwan National Health Insurance Research Database were enrolled in this study. The date of diagnosis of achalasia and the date of diagnosis of ESCC were defined as the index date. The ESCC cohort consisted of 462 achalasia patients (ICD-10: ICD-10: C16) who were enrolled in the 1996-2005 period of time. The cancer-free cohort comprised of 3,091,117 subjects identified from the 2001 Registry for Catastrophic Illness Patient Database as the control cohort. The incidence density rate of ESCC for achalasia patients was 2.13 times higher than that for control subjects (95% confidence interval [CI]: 1.79, 2.54). In addition, the cumulative proportion of the patients with ESCC was 1.5% higher in the achalasia cohort than in the control cohort. The subgroup analysis showed that the highest incidence rate of esophageal cancer was observed at year 2005 (1.23, 95% CI: 0.99, 1