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The French registry for glycogen storage disease type 3 was established in 2014, with the aim of prospectively and nationally collecting epidemiological, genetic, muscular, cardiac, and metabolic data from patients. 72 patients were included (43F/29M). The mean age at molecular diagnosis was 16 years [0-60], and the mean age at registry inclusion was 23 years [4-65]. 60 patients underwent muscular evaluation, with an initial assessment on average at 26 years. Among patients under 18 years old (n=21), the mean Motor Function Measure (MFM) scores were: D1 93.62% [66.67-100], D2 98.94% [83.33-100], D3 95.92% [85.71-100], Total 96.12% [83.33-100]. The mean time to climb 4 steps was 2.03 s [1.31-3.21], run 10 meters was 3.62 s [2.95-4.75], and the 6-minute walk test was 505m [350-700]. 3 patients had walking difficulties. Mean CK levels was 1403 [34-4163]. Among patients aged 18 years and older (n=39), the mean MFM scores were: D1 74.35% [2.56-100], D2 92.66% [50-100], D3 95.73% [66.67-100], Total 84.98% [32-100]. The mean time to climb 4 steps was 3.64 s [1.56-18.34], run 10 meters was 9.16 s [2.66-102], and the 6-minute walk test was 467m [50-677]. 21 patients had walking difficulties. Mean CK level was 2569 [52-12056]. 29 patients developed left ventricular hypertrophy on echocardiography, 31 on ECG, and 29 patients had cardiac repolarization disorders. For a rare disease like glycogen storage disease type 3, the existence of registries is essential. We demonstrate here that muscular involvement worsens with age, and cardiac involvement is often present, albeit moderate. [ABSTRACT FROM AUTHOR]