While cholestatic jaundice usually develops in the advanced stages of primary biliary cirrhosis, our series of this disease disclosed that 8 of the 88 cases presented cholestatic jaundice in the early histologic stages (stages 1 and 2). These patients frequently presented esophageal varices (57%) and showed a low incidence of positive mitochondrial antibodies compared to the non-jaundices cases in the same histologic stages. Histologic changes of livers from the jaundiced patients failed to show fundamental differences from those of the non-jaundiced patients, except for the presence of bile plugs and extensive bile duct loss in the former. Deposition of orcein-positive granules and increased hepatic copper content, suggesting prolonged cholestasis, were rather advanced, and inflammatory changes in the portal tracts and piecemeal necrosis were rather mild in the jaundiced patients. These data suggest that cholestatic jaundice occurs in a few patients in the early histologic stages on a biopsy with extensive bile duct loss and features of prolonged cholestasis; it is not clear whether such patients are a small separate cluster in PBC, or not.