Primary Biliary Cirrhosis (PBC) is a debilitating autoimmune condition associated with a constellation of extrahepatic inflammatory manifestations accentuating its morbidity and mortality. Pericarditis and pericardial effusion had been rarely associated with and inadequately characterized in PBC. We present a challenging case of inflammatory pericarditis and life-threatening massive pericardial effusion in a 49 year old patient with PBC. This complication should be more recognized and appropriately treated to avoid progression to cardiac tamponade.