Objective: To summarize the characteristics of arrhythmia with the relevant factors affecting its prognosis in patients of apical hypertrophic cardiomyopathy (AHCM). Methods: A total 283 AHCM patients with echocardiography or cardiac magnetic resonance (CMR) conifrmed diagnosis in our hospital from 2005-01 to 2012-08 were summarized. The patients were divided into 2 groups: With arrhythmia group, n=103 and Without arrhythmia group,n=180. The endpoint event was followed-up by clinical and telephone visits in both groups and the relevant risk factors affecting AHCM prognosis were investigated by Cox regression analysis. Results: There were 269 patients ifnished the follow-up investigation, 98 in With arrhythmia group, 171 in Without arrhythmia group, and the death rate was 4.08% vs 1.17%, the occurrence of endpoint event was 18.37% vs 5.58%respectively. Cox regression analysis indicated that age (HR=23.051, 95% CI 1.08-1.068,P<0.005), left atrial diameter (HR=4.113, 95%CI 1.002-1.119,P=0.043) and NT-proBNP (HR= 18.653, 95% CI 3.433-26.650,P<0.005) were the independent risk factors affecting prognosis in AHCM patients. Conclusion: Arrhythmia is one of the common presentations of AHCM, it does not have much impact on survival, while it may cause ventricular ifbrillation and increase the occurrence of cardiovascular events.