Pylephlebitis occurs secondary to infection in the region drained by the portal venous system. We described a case of pylephlebitis which developed as a complication of appendicitis and reviewed 22 cases of pylephlebitis, including the current case, reported in Korea. Appendicitis, followed by diverticulitis, was the most common infection associated with pylephlebitis. Fever and abdominal pain were the most common presenting symptoms. Bacteremia was present in 60% of cases. Streptococci, enteric gram-negative bacilli, and Bacteroides fragilis were common organisms isolated from the blood. Liver abscess was identified in 3 cases. All patients survived regardless of anticoagulation therapy. Although pylephlebitis is a rare disease, it should be included in the differential diagnosis in patients with fever of unknown origin who present with nonspecific abdominal symptoms or abnormal liver function test.