We aimed to assess the relative contribution of genes and environment in the aetiology of Dupuytren’s disease by studying Danish twins born between 1870 and 2000. Twins with a diagnosis ( n = 365) and the subgroup who also had an operation ( n = 259) after 1977 were identified through linkage with a nationwide hospital registry among 30,330 monozygotic and same-sexed dizygotic twin pairs. Since monozygotic twins share all their genes and dizygotic twins share on average half of their genetic material, greater phenotypic similarity is expected in monozygotic than in dizygotic twins if a genetic component is involved. The number of concordant male twin pairs with Dupuytren’s disease was 17 and 7 (monozygotic and dizygotic pairs, respectively), compared with 60 and 174 discordant monozygotic and dizygotic pairs, yielding probandwise concordance rates of 0.37 (95% confidence interval (CI): 0.26 to 0.50) and 0.07 (95% CI: 0.04 to 0.14), respectively. The heritability of Dupuytren’s disease was approximately 80%. We conclude that genetic factors play a major role in the development of Dupuytren’s disease. Level of evidence: 3