OBJECTIVE Arterioportal and arteriovenous fistulas (APF, AVF) are rare vascular disorders occurring as a result of congenital vascular malformation, trauma, iatrogenic causes or neoplasms. The clinical spectrum of presentation ranges from symptom-free individuals to patients with severe portal hypertension or congestive heart failure. While the majority of patients have been treated surgically in the past, interventional radiological procedures are being performed with increasing frequency. To prove this trend we reviewed the international literature. METHODS We reviewed the clinical presentation and management of 79 cases reported between 1980 and 1996 in the literature and six own patients. RESULTS Review of the literature and our own six cases show that these fistulas can be divided in intrahepatic (n = 49, 75%) and extrahepatic fistulas (n = 20, 25%). The most important causes are trauma (n = 29, 37%), iatrogenic induced by procedures (n = 21, 27%) and congenital vascular malformations (n = 10, 13%). APFs and AVFs can be diagnosed by ultrasound, computed tomography and magnetic resonance imaging. Angiography confirms the diagnosis and in many cases allows definitive interventional radiologic treatment. CONCLUSIONS Management of AVFs and APFs remains a challenge. However, interventional radiologic procedures provide a safe, low-cost, and effective method for treatment. Due to these facts in the last three years we notice an increasing part of interventional procedures for treatment.