Splenic rupture in neonates is a very rare problem that may result in life threatening blood loss. The main causes of splenic rupture in the neonatal period include traumatic rupture, erythroblastosis fetalis, coagulopathy and vascular pathologies of the spleen. The diagnosis is difficult, may be delayed and is sometimes only revealed on autopsy due to the non-specific symptoms – unexplained anaemia and abdominal distension. High suspicion of possible haemorrhage in the early stage of the disease, exploration of the source on abdominal ultrasound and, when needed, immediate surgical intervention to control blood loss, are the cornerstones of successful therapy. We describe a case of splenic rupture associated with the haemolytic disease of the newborn and complicated by haemorrhagic shock in a one-day old neonate. Splenectomy was performed and the child survived.