Patients with apparent polycythaemia are characterised by a raised packed cell volume (PCV; males above 0.51, females above 0.48) but normal red cell mass (RCM; less than 25% greater than predicted). Prediction and interpretation of RCM and PV should be based on height and weight, since the use of body weight alone is misleading. Patients with PCV values up to 0.60 may have apparent polycythaemia but only 18% have a reduced PV (relative polycythaemia). Therefore, the most common cause of the raised PCV is a change in RCM and/or PV within their normal ranges. The clinical associations and possible causes for the RCM/PV changes include male sex, obesity, dehydration, diuretics, smoking, hypertension, alcohol, arterial oxygen desaturation, renal disease and increased catecholamine levels. Retrospective studies of patients with apparent polycythaemia and information from other groups of polycythaemic patients suggest an increased risk of vascular occlusion, although other factors, such as hypertension and smoking, are also involved. Proposed management includes modification of possible underlying causes and examination for risk factors for vascular occlusion. In patients with PCV levels chronically above 0.54 venesection should be used, but patients with PCV values below this level should only be venesected if they are considered to be at risk of vascular occlusion. The suggested target value for PCV for venesected patients is 0.45 or below.