This case report describes the peri-operative course of a patient with uncontrolled polycythaemia vera who underwent a laparoscopic hepatectomy for intrahepatic cholangiocarcinoma. Polycythaemia vera is a chronic condition that results in erythrocytosis and puts patients at risk of peri-operative complications including thrombotic events and paradoxical haemorrhage. Little evidence exists on the ideal peri-operative management of uncontrolled polycythaemia vera when the proposed procedure carries a high risk of haemorrhage. Our patient presented with a pre-operative haemoglobin of 197 g.l À1 (haematocrit 65%) and was not phlebotomised pre-operatively. Intra-operatively he lost 2700 ml of blood, reducing his haematocrit to 48%, and then suffered fatal thrombotic complications postoperatively. The patient did not receive any blood product transfusions during his peri-operative course. We review the available evidence to guide the perioperative management of patients with polycythaemia vera. The inherent risks of thrombosis and haemorrhage associated with polycythaemia vera need to be weighed against the specific surgical and transfusion-related risks. Phlebotomy to achieve a pre-operative haematocrit under 45% is recommended and intra-operative phlebotomy shows promise for reducing blood loss during hepatectomies. Management of postoperative erythrocytosis may be an important and underappreciated aspect of reducing the peri-operative risk of thrombosis in patients with polycythaemia vera.