Primary biliary cirrhosis (PBC) and sarcoidosis are both granulomatous disorders of unknown etiology. Although they are distinct clinical entities, a small number of patients presenting with manifestations of both has been reported. We describe a female patient with PBC and sarcoidosis who developed a malignant cutaneous tumor with histologic and immunohistochemical characteristics of a sweat gland carcinoma (SGC). An SGC, developing in a patient with concurrent PBC and sarcoidosis has not been reported previously.