BACKGROUND: Jackhammer esophagus (JE) is a newly described esophageal motility disorder. However, there are limited data on JE and this entity remains misunderstood. METHODS: We performed a systematic review of clinical series on JE through MEDLINE, EMBASE, and Web of Science. Data from included studies were then extracted, and random-effects meta-analyses were performed. RESULTS: Thirty-eight studies met inclusion criteria. The pooled prevalence of JE was 1.97% [95% CI: 1.39%-2.78%] among patients referred for high-resolution manometry (HRM). The mean age at diagnosis was 60.8 years [95% CI: 57.1-64.4] and 65% [95% CI: 58%-72%] of patients were female. JE was significantly more prevalent in postlung transplant patients and morbidly obese patients undergoing HRM (23.99% [95% CI: 9.00%-38.97%] and 5.18% [95% CI: 1.76%-14.3%] respectively). Dysphagia was the most common presentation of JE (64% [95% CI: 52%-77%]) followed by gastroesophageal reflux disease (46% [95% CI: 26%-67%]). The pooled mean distal contractile integral (DCI) of all standard HRM swallows was 9249 mm Hg·s·cm [95% CI: 7834-10 663], and the pooled mean integrated relaxation pressure (IRP) was 13.9 mm Hg [95% CI: 8.2-19.7]. Overall, 73.6% [95% CI: 64.0%-83.1%] of JE patients who underwent treatment achieved clinical symptom improvement (79% [95% CI: 74%-85%] for endoscopic treatment and 63% [95% CI: 47%-79%] for medical treatment). The pooled clinical success rate of peroral endoscopic myotomy (POEM) specifically was 82% [95% CI: 75%-90%]. CONCLUSIONS: JE is a new motility disorder most commonly presenting with dysphagia. High clinical suspicion is important because the diagnosis can only be made through HRM.