Summary. Severe haemophilia and reduced bone density can negatively influence perception of patient’s health-related quality of life (HRQoL), especially considering future aspects, the risk of losing independence or pain suffering. The aim of this study was to assess levels of HRQoL in severe haemophilia patients and to compare HRQoL to those of the general population as well as to determine whether reduced bone density is correlated to the perceived HRQoL. Patients were divided into two groups based on timing of being treated with prophylaxis: Group A (started prophylaxis at age of ≤3 years; n = 22); Group B (at age of >3 years; n = 15). The bone mineral density (BMD g cm−2) of different measured sites was measured by dual energy X-ray absorptiometry (DXA). HRQoL was assessed using SF-36 questionnaire. Group A have mean BMD T-score >−1.0 (i.e. normal score) at all measured sites, and have almost similar scores in the SF-36 domains compared with the reference population. Group B have mean BMD T-score <−1.0 at hip region, and >−1.0 at lumbar spine and total body, and their scores in the SF-36 domains were lower compared with the reference population. Moreover, significant correlations were found between BMD at femoral neck and total body with physical domains. With adequate long-term prophylaxis since early childhood, adult patients with haemophilia report a comparable BMD and HRQoL to the Swedish reference population. Reduced BMD in group B correlated with impaired physical health, which underscores the importance of early onset of adequate prophylactic treatment.