OBJECTIVES: Hereditary haemochromatosis (HH) is a common autosomal recessive disorder amongst persons of European heritage. Elevated hepcidin production increases the absorption of dietary iron, which is stored in the parenchymal tissues of the heart, liver and pancreas. Treatment consists of regular venepuncture, and if implemented early, prevents organ damage.   If untreated, iron overload can be a cause of morbidity and mortality. To date, a lack of robust health economic evidence has been cited as one of the barriers to establishing screening programs for HH. Previous analyses have used unvalidated estimates of health state utility values (HSUV). This is in part due to no published estimates of HSUV. This study sought to estimate HSUV directly from people with different levels of severity of HH of in Australia. METHODS: Volunteers with HH were recruited to complete a web-based survey as part of a national cost of illness study for HH. HSUV was estimated using the Assessment of Quality of Life 4D (AQOL-4D) instrument. Severity of HH was categorised according to the method recommended by the European Association of the Study of the Liver. Multivariate regression analysis was performed to identify parameters associated with HSUV. RESULTS: Between November 2013 and November 2014, 221 people completed the survey.  Increasing severity of HH was negatively associated with HSUV. Mean (standard deviation) HSUV were 0.76 (0.21), 0.81 (0.18), 0.60 (0.27), and 0.50 (0.27) for grades 1-4 HH respectively. CONCLUSIONS: Increasing severity of HH is associated with decreasing HSUV. Previous cost utility analyses have used higher HSUV which likely resulted in underestimates of the cost effectiveness of screening programs for HH. The HSUV reported in this paper are the most robust available and their use would improve the validity of future economic models for HH.