Budd-Chiari syndrome (BCS) and constrictive pericarditis (CP) share many common clinical features. Over the last year we encountered three patients in whom CP clinically mimicked BCS. Two of the three did not even have raised jugular venous pressure. One patient with severe jaundice and hepatic coma ultimately died. Liver biopsy features were not discriminating. The final diagnosis of CP was established by echocardiography, chest computed tomography (CT), or cardiac catheterization. We conclude that in all patients with apparent BCS and atypical features, a noninvasive test like echocardiography or chest CT should be done to rule out treatable illness like CP before embarking on such invasive procedures as liver biopsy for diagnosis.