AbstractBackground & AimsBudd‐Chiari syndrome (BCS) is a rare and potentially life‐threatening disorder characterized by obstruction of the hepatic outflow tract. It is unknown whether patients with BCS represent a high risk for severe disease and mortality from coronavirus disease 2019 (COVID‐19). Thus, we aimed to assess hospitalization rates, severe disease, all‐cause mortality, intensive care unit (ICU) requirement and acute kidney injury (AKI) from COVID‐19 diagnoses.Methods & ResultsWe identified 467 patients with BCS with COVID‐19, 96 427 non‐chronic liver disease (CLD) and 9652 non‐BCS CLD. The BCS and non‐CLD cohorts (n = 467 each) and BCS and non‐BCS CLD (n = 440 each) were well balanced after propensity matching. When compared to the non‐CLD cohort, the BCS group had a higher risk of all‐cause mortality (5.1% vs. 2.4%, HR 2.18; 95% CI, 1.08–4.40), severe disease (6.0% vs. 2.4%, HR 2.20; 95% CI, 1.09–4.43), hospitalization (24.6% vs. 13.1%, HR 1.77; 95% CI, 1.30–2.42) and AKI (7.9% vs. 2.8%, HR 2.57; 95% CI, 1.37–4.85), but no significant differences in ICU requirements (2.4% vs. 2.1%, HR 0.75; 95% CI, 0.27–2.08) at 60‐days time points. When compared to the non‐BCS CLD cohort, the BCS group had a higher risk of all‐cause mortality (3.6% vs. 2.5%, HR 3.94; 95% CI, 1.31–11.79), hospitalization (29.8% vs. 21.6%, HR 1.43; 95% CI, 1.09–1.86), but differences in ICU requirements (HR 0.90 (0.38–2.12)), AKI (HR 1.41 (0.86–2.30)) or severe disease (HR 1.92 (0.99–3.71)) did not reach statistical significance at 60‐day follow up.ConclusionIn conclusion, COVID‐19 infection in patients with BCS is associated with poor outcomes. Patients with BCS infected with COVID‐19 carry a significantly higher risk of hospitalization and all‐cause mortality and a possible effect on severe disease and AKI compared with COVID‐19 patients without CLD or with non‐BCS‐CLD.