Pregnancy in a woman with paroxysmal nocturnal haemoglobinuria was complicated by hepatic venous thrombosis, portal hypertension and haematemesis due to gross oesophageal varices. Labour was induced and a live female infant was born. Dural sinus thrombosis and intracerebral haemorrhage resulted in death on the 46th day post partum. A review of the literature shows that venous thrombosis is common in paroxysmal nocturnal haemoglobinuria.