Retroperitoneal arteriovenous malformations (AVMs) are rare lesions consisting of dysplastic vessels, which may produce diverse clinical features. We report a case of a neonate born on the 29th week of gestation, who presented with heart failure, generalized oedema, respiratory dyspnoea and metabolic acidosis. Digital subtraction angiography revealed a large AVM located adjacent to the aorta and the inferior vena cava. Lumbar arteries supplying the AVM were occluded by coils. The AVM was partially embolized using N-butyl cyanoacrylate. Despite the apparently successful embolization procedure, heart failure and respiratory distress progressed. The newborn died on her fourth day of life. We report the first case describing AVM embolization in a very low birth weight preterm infant.