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Background Wilson Disease (WD) is a rare genetic disorder of copper metabolism. Without appropriate treatment it can progress to liver failure and death. The National Congenital Anomaly and Rare Disease Registration Service (NCARDRS), with support from the Wilson Disease Special Interest Group, has established registration of WD in England. We aim to provide a descriptive study of mortality, including multiple cause of death and transplant status, of those with WD. Method Confirmed cases of WD were reported by 20 hospital trusts and registered with NCARDRS enabled by their legal permissions (CAG 10-02(d)/2015) to collect patient data without consent. Vital status of all cases were determined and linkage with Office of National Statistics (ONS) mortality data was undertaken to obtain death certificate data. Cases of E83.0 Disorders of copper metabolism, between 2008–2018, were extracted from ONS mortality data. Cause of death free text was manually searched to identify deaths that mentioned WD. All deaths were linked to Hospital Episode Statistics (HES) inpatient data to determine transplant status. Results Death records were identified for 52 patients, 65% were male, with a mean age of 45.5 years (range 17–82). Complications related to cirrhosis or liver failure were assigned as the underlying cause of death (UCOD) in 44%. Hepatocellular carcinoma (HCC) was the UCOD in 5.8%. Of the 21% of patients who were recorded as having a liver transplant, transplant complications or graft failure were recorded as a cause of death in 8%. Sepsis was mentioned on the death certificate in 42% and recorded as the UCOD in 21%. Conclusion The contribution of WD to mortality in England will be underestimated unless multiple cause of death analysis is undertaken. The number of deaths resulting from complications related to cirrhosis or liver failure suggests that there might be missed opportunity for liver grafting. HCC was the cause of death in 5.8% of cases suggesting the prevalence of HCC in WD may be higher than previously thought. This project demonstrates the utility of the NCARDRS’ for WD in England.