BACKGROUND: Although rare, portal mesenteric venous thrombosis and pylephlebitis remain potential life-threatening sequelae of ruptured appendicitis in children. Treatment recommendations from recent reports have included urgent exploratory laparotomy with appendectomy, prolonged intravenous antibiotic therapy, and anticoagulation for up to a year. METHODS: This report describes successful management of pylephlebitis and mesenteric venous thrombosis complicating ruptured appendicitis with intravenous antibiotics and anticoagulation followed by interval laparoscopic appendectomy. RESULTS: A previously healthy 5-year-old girl was diagnosed with ruptured appendicitis complicated by pylephlebitis and mesenteric venous thrombosis at the time of presentation. She was treated with intravenous antibiotics and anticoagulated for 3 months. She subsequently underwent interval laparoscopic appendectomy. At 3-year follow-up, she is healthy without evidence of adverse sequelae. DISCUSSION: This is the first reported case of successful, minimally invasive management of ruptured appendicitis complicated by mesenteric venous thrombosis and pylephlebitis. CONCLUSION: Similar treatment of other children with this rare presentation seems reasonable.