A 53-year-old woman with concurrent systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC) developed a deforming but minimally symptomatic arthropathy. The simultaneous occurrence of SLE and PBC is believed to be rare. Moreover, destructive arthritis, in the absence of serum rheumatoid factor, has not been previously reported with either disease. We review the association of liver disease with arthritis and propose a possible mechanism to account for the severe joint destruction seen in our patient.