Budd-Chiari syndrome is characterized by hepatic venous outflow tract obstruction. We describe an 18-year-old female of known celiac disease presented with progressive abdomen distention and shortness of breath for the last 1 month. Computed tomography of abdomen revealed hepatic vein obstruction. The patient was diagnosed with Budd-Chiari syndrome. Coagulation profile showed an increased homocysteine level. Serum folate level was also decreased. The patient was put on oral anticoagulant with a gluten-free diet. After 4 weeks, the patient showed significant improvement with decreased ascites. The association of Budd-Chiari syndrome with Celiac disease has not yet been fully understood. There have been few reports that described this rare association. Budd-Chiari syndrome should be considered as an important differential in a patient with unexplained ascites and celiac disease.