Autoimmune cholangitis (AC) is a recently proposed entity that describes a specific group of patients presenting overlapping features of primary biliary cirrhosis (PBC) and autoimmune hepatitis. The disease is characterized by dinical cholestasis, high titer antinuclear antibody, negative antimitochondrial antibody, and histologically, findings of PBC coexisting with varying degrees of parenchymal inflammation. In this report, we describe a patient with Sjogren's syndrome who fulfilled the diagnostic criteria of AC associated with unique arthropathy compatible with arthritis of PBC. This case illustrates the unusual coexistence of two diseases that may share similar pathogenic processes.