Loin pain haematuria syndrome is a descriptive diagnosis of recurrent episodes of loin pain accompanied by haematuria, in which investigations do not reveal adequate pathology to account for the symptoms. The majority of patients present between 20 and 40 years, but onset may occur in older children. A significant number of patients show psychological and psychopathological features. Renal histology may show minor abnormalities, including mesangial proliferation, arteriolar and arterial hyalinosis and C3 in arterioles. Renal angiography is often normal but changes in intrarenal arterioles and cortical infarcts may be seen. Haematological abnormalities include decreased heparin-thrombin clotting time and elevated free plasma serotonin concentration. It is important that the assessment include a detailed psychiatric history, the patient's perception of pain, and the psychosocial environment. The pain may be very severe, leading to the requirement for addictive analgesics: management often becomes very difficult and frustrating to medical practitioners. Surgical intervention with capsulotomy, denervation and autotransplantation should only be considered as a last resort, as there is frequent recurrence of pain on the same or contralateral side.