Pylephlebitis is defined as thrombophlebitis of the portal vein or its tributaries. It is a rare disease commonly associated with acute appendicitis and it has a high rate of morbidity and mortality. Doppler ultrasound and computed tomography scan are the methods of choice to confirm diagnosis. The most frequent long-term complication in these patients is portal vein cavernoma, with subsequent portal hypertension. An early diagnosis, timely antibiotic therapy and removal of infection are essential therapeutic measures to diminish mortality. The need for anticoagulation therapy in children remains controversial. We report on a child with acute abdomen and sepsis with a diagnosis of pylephlebitis secondary to appendiceal inflammation. The patient received long-term antibiotic therapy and subcutaneous anticoagulation. Deferred appendectomy was performed with favorably outcome.