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Primary Biliary Cholangitis (PBC) is a debilitating autoimmune disorder involving intrahepatic biliary ducts. It is characterized by chronic inflammation followed by fibrous obliteration of biliary ducts. This condition is rarely associated with pericardial effusion. The association of pericarditis and PBC is rare and identifies the existence of extensive systemic inflammation. In the literature, only 5 cases of this association are reported. As a specific systemic syndrome, this association should be evaluated and its prognostic consequences should be analyzed in depth. We report a case of life-threatening pericardial effusion progressing to tamponade with pericarditis in a 38-year-old female patient with PBC.