Summary Prophylaxis is the treatment of choice for children with severe haemophilia. As prophylaxis is especially important during the period of growth, the need for continued prophylaxis in adulthood should be considered. The aim of this study was to describe the incidence and outcome of stopping prophylaxis in patients with severe haemophilia who were offered prophylaxis during childhood. All patients with severe haemophilia (factor VIII/IX <0·01 IU/ml), born 1970–80, treated in two Danish and one Dutch treatment centre were studied. Data on discontinuation of prophylaxis, treatment, joint bleed frequency, clinical scores and radiological scores were collected. Eighty patients were studied. Median follow‐up was 19 years (range 7–29). A total of 35% of patients discontinued prophylaxis at a median age of 21·5 years [interquartile range (IQR) 18·4–24·4], experiencing only three joint bleeds per year (IQR 1·4–8·7). Median clinical scores were similar in patients who discontinued prophylaxis [4 points (IQR 0–6)] and those who continued [3 points (IQR 1–6)], as were median Pettersson scores at 13 (IQR 1–24) vs. 13 points (IQR 5–23) respectively. In conclusion one‐third of young adults with severe haemophilia on a prophylactic regimen discontinued prophylaxis in early adulthood, while maintaining a low joint bleed frequency and similar arthropathy after 4 years.