We report a case of primary sclerosing cholangitis (PSC) with benign lyphadenopathy which was diagnosed with endosonography guided fine needle aspiration (EUS-FNA). A 65-year-old woman was admitted to Jikei University Hospital with severe jaundice. Although endoscopic retrograde cholangiography and liver biopsy revealed the findings consistent with PSC, abdominal computed tomography revealed numerous large perihepatic lymph nodes with a maximum diameter of more than 3 cm. Therefore, EUS-FNA was done in order to exclude malignant lymphadenopathy, and adequate specimens obtained by EUS-FNA showed reactive hyperplasia of lymphnode. The patients were scheduled to undergo liver transplantation. We report a case of primary sclerosing cholangitis (PSC) with benign lyphadenopathy which was diagnosed with endosonography guided fine needle aspiration (EUS-FNA). A 65-year-old woman was admitted to Jikei University Hospital with severe jaundice. Although endoscopic retrograde cholangiography and liver biopsy revealed the findings consistent with PSC, abdominal computed tomography revealed numerous large perihepatic lymph nodes with a maximum diameter of more than 3 cm. Thus, EUS-FNA was done in order to exclude malignant lymphadenopathy, and the satisfied specimens obtained by EUS- FNA showed reactive hyperplasia of lymphnode. The patients were scheduled to undergo liver transplantation.