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Background Both early repolarization and altered heart rate profile are associated with sudden death. In this study, we aimed to demonstrate an association between early repolarization and heart rate profile during exercise.Methods A total of 84 subjects were included in the study. Comparable 44 subjects with early repolarization and 40 subjects with normal electrocardiogram underwent exercise stress testing. Resting heart rate, maximum heart rate, heart rate increment and decrement were analyzed.Results Both groups were comparable for baseline characteristics including resting heart rate. Maximum heart rate, heart rate increment and heart rate decrment of the subjects in early repolarization group had significantly decreased maximum heart rate, heart rate increment and heart rate decrement compared to control group (all P＜0.05). The lower heart rate increment (＜106 beats/min) and heart rate decrement (＜95 beats/min) were significantly associated with the presence of early repolarization. After adjustment for age and sex, the multiple-adjusted OR of the risk of presence of early repolarization was 2.98 (95% CI 1.21-7.34) (P=0.018) and 7.73 (95% CI 2.84-21.03) (P ＜0.001) for the lower heart rate increment and heart rate decrement compared to higher levels, respectively.Conclusions Subjects with early repolarization have altered heart rate profile during exercise compared to control subjects. This can be related to sudden death.