Background Medical treatment plays a main role in the treatment of Jackhammer esophagus (JE). The efficacy of calcium channel blockers (CCBs) combined with proton pump inhibitors (PPIs) in the treatment of JE has not been established. Methods This 8-week randomized, open-label study was conducted in an outpatient setting in China. Patients with symptomatic JE were randomly assigned to receive diltiazem combined with lansoprazole (DL group) or diltiazem alone (D group). The primary outcome was the response rate after 8 weeks. Secondary outcomes included symptoms, quality of life, manometric pattern and reflux status. Key Results Forty patients were randomized, 38 (95.0%) of whom completed the study (20 patients in the DL group, and 18 were randomized to the D group). A higher response rate was found in the DL group than in the D group (85.0% vs. 55.0%, p= 0.038). All secondary outcomes, including overall symptom control, quality of life, and reflux control, were significantly better in the DL group than in the D group after 8 weeks (p<0.05), except the distal contractile integral (DCI) (p=0.274). Conclusions Diltiazem combined with lansoprazole is superior to diltiazem alone in obtaining treatment response and correcting abnormal reflux situations in patients with symptomatic JE.