Umbilical artery catheterisation is frequently performed in preterm or critically ill newborn infants for invasive monitoring of blood pressure and blood sampling for laboratory tests. It is associated with well-known complications like catheter-related infections, thromboembolic events and aneurysmal formation of the aorta. In this report, we present another major complication of umbilical artery catheterisation: Umbilical artery perforation. This complication occurred in a prematurely born infant and resulted in severe haemorrhagic shock, subsequent renal failure and severe periventricular leukomalacia. Ultimately, the patient deceased 3 weeks after birth. Review of the literature identified only a few case reports and retrospective postmortem studies describing this complication. Neonatologists and paediatricians performing umbilical artery catheterisation should be aware of this rare, but potentially life-threatening complication.