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Introduction Long-term weight change since young-adulthood is a better indicator of distribution of body fat mass. We investigated weight change from age 20 in relation to the risk of CVD mortality, including specific causes of CVD, using data of a large population-based cohort study in Japan. Methods In 1994, 41 364 eligible individuals aged 40–79 years responded to the questionnaire survey. Participants were categorised by weight change: loss ≥10 kg and 5−9.9 kg, ±4.9 kg, gain 5−9.9 kg and ≥10 kg. During the follow-up period of 13.3 years, 1756 deaths of CVD were identified and risk analysis was done by Cox proportional hazards model. Results For all CVD mortality in men were significantly increased for weight loss more than 10 kg with the HR 1.52. Weight gain didn9t predict increased risk in our study. In women, U-shaped association was found while the HRs were 1.64 and 1.35 for those with 10 kg weight loss and weight gain, respectively. The risks for coronary and stroke mortality were also increased for women who lost their weight with the HRs 2.03 and 1.49. The risk trends were similar in subgroup analysis of elderly, non-smokers and early death deletion. Conclusion Weight loss since early adulthood was a predictor of death from CVD. Weight gain more than 10 kg was associated with increased risk of CVD mortality among women.