This report describes a case of portal hypertension caused by periportal tuberculous lymphadenitis. There were a few reports of portal hypertension associated with tuberculosis. A 27-year-old man was admitted to the hospital because of recurrent hematemesis for 7 days. There was a history of mediastinal tuberculous lymphadenitis 3 years earlier that was treated with isoniazide, rifampin, ethambutol, and pyrazinamide for 2 years. Clinical evaluation revealed esophageal variceal bleeding and main portal vein obstruction by enlarged periportal lymph nodes. The patient underwent distal splenorenal shunt. Pathologic examination of the excised periportal lymph node revealed chronic granulomatous inflammation with central caseous necrosis. Thereafter the patient took antituberculous medication for 12 months. The patient has not re-bled 3 years since the shunt operation.