•Maximal knee extensor muscle strength increased after 12 weeks of strength training.•Walking speed increased after training and was maintained at month 9.•Standing up capacity increased after training and was maintained at month 9.•Muscle fiber diameter did not significantly change after training.•Abnormal hypertrophic factor at baseline could influence fiber growth. Myotonic dystrophy type 1 (DM1) is a multisystemic disease characterized by progressive muscle weakness. The aim of this project is to evaluate the effects of a 12-week lower limb strength training program in 11 men with DM1. Maximal isometric muscle strength, 30-second sit-to-stand, comfortable and maximal 10-m walk test (10 mwt) were evaluated at baseline, 6 and 12 weeks, and at 6 and 9 months. The one-repetition maximum strength evaluation method of the training exercises was completed at baseline, 6 and 12 weeks. Muscle biopsies were taken in the vastus lateralis at baseline and 12 weeks to evaluate muscle fiber typing and size (including atrophy/hypertrophy factors). Performance in strength and functional tests all significantly improved by week 12. Maximal isometric muscle strength of the knee extensors decreased by month 9, while improved walking speed and 30 second sit-to-stand performance were maintained. On average, there were no significant changes in fiber typing or size after training. Further analysis showed that individual abnormal hypertrophy factor at baseline could explain the different changes in muscle size among participants. Strength training induces maximal isometric muscle strength and lasting functional gains in DM1. Abnormal hypertrophy factor could be a key component to identify high and low responders to hypertrophy in DM1.