Sarcoidosis and primary biliary cirrhosis (PBC) are thought to be two distinct disorders of unknown origin. However, both are characterized by hepatic granuloma formation and may also manifest cutaneous granulomatous inflammation. In this report, we describe two cases of cutaneous sarcoidosis occurring in the setting of PBC and review 7 additional cases from the literature of granulomatous skin disease associated with PBC. Although the pathogenesis of both sarcoidosis and PBC remains elusive, the simultaneous occurrence of these uncommon diseases suggests a common pathway may contribute to granuloma formation in both disorders.