Background and AimThis aims to study incidence of re‐bleeding on anticoagulation and survival of Budd–Chiari syndrome (BCS) patients presenting with variceal bleeding.MethodsBudd–Chiari syndrome patients presenting with variceal bleed between 01/01/2007 and 01/05/2019 were retrospectively studied. Patients underwent endoscopic treatment ± endovascular therapy, followed by anticoagulation. Variceal re‐bleed (on anticoagulation) and survival were studied.ResultsOf 376 BCS patients diagnosed during the study period, 40 (10.7%) patients, presenting with variceal bleed (age 33 [25–40] years; male patients 70%; Rotterdam score 1.13 [0.63–1.22]), Group 1 were compared with 40 randomly selected age‐matched BCS patients presenting with ascites, no bleeds (40 [23–42] years; male patients 42.5%; Rotterdam score 1.11 [1.09–1.16]), Group 2. The commonest site of obstruction was hepatic vein (65%) in Group 1 and combined hepatic veins and inferior vena cava (57.5%) in Group 2 (P < 0.01). Thirty‐six Group 1 patients underwent endoscopic intervention (variceal ligation, 33; sclerotherapy, 2; glue injection, 1). Endovascular intervention was performed in 30 Group 1 patients (angioplasty ± stent, 22; endovascular shunt, 8) and in 34 Group 2 patients (angioplasty ± stent, 26; endovascular shunt, 8). All 80 patients were started on anticoagulation. Variceal bleed on anticoagulation occurred in five patients in Group 1 and three patients in Group 2. One‐year and 5‐year survival were 94.2% and 87.5%, respectively, in Group 1 and 100% and 80%, respectively, in Group 2.ConclusionsAbout one‐tenth of BCS patients present with variceal bleed. On management with endoscopic ± endovascular therapy, followed by anticoagulation, variceal re‐bleed in these patients were comparable with those in BCS patients presenting with ascites and survival was excellent at 1 and 5 years.