Primary sclerosing cholangitis (PSC) is a cholestatic disease characterized by segmental narrowing and dilatation of bile ducts. Few studies have been performed on the laparoscopic findings associated with this disease, and the present study was intended to assess the usefulness of laparoscopy for the diagnosis and staging of PSC.Six patients were examined by laparoscopy with liver biopsy. Repeated laparoscopy was performed in three patients.Laparoscopy revealed coarse surface irregularity and discoloration. Surface irregularity was classified into four grades: smooth, shallowly depressed, undulated, and nodular. The affected area showed whitish yellow discoloration. The discolored area was demonstrated as a poorly stained area by intravenous injection of indocyanine green (ICG). Lobular markings became apparent because of the yellow color change in the portal tract, resulting in a leopardskin-like appearance. Lymph-vessel dilatation was seen in advanced stages. Repeated laparoscopy of a patient without treatment demonstrated a progression from a smooth surface to a shallow depression with leopardskin-like markings. On the other hand, the two patients treated with immunosuppressive agents showed improvement of liver swelling and disappearance of the leopardskin-like markings and lymph-vessel dilatation.Laparoscopy may provide useful information for the diagnosis and staging of PSC.