Visible or non-visible haematuria could represent a range of urological or nephrological diseases, or bear no significance at all. The challenge for clinicians is to get the correct patients assessed rigorously so that life-altering or life-limiting disease is not missed. Up-to-date definitions and epidemiological data is discussed. This article provides a clear framework to help decide which patients have significant haematuria and which patients are suitable for urological referral; a decision algorithm is also included. We discuss how to investigate such patients further, including points not to forget when taking a history or conducting physical examination. Explanations are provided as to why current management strategies exist and what the potential flaws are of each method, including the ubiquitous urine dipstick test, urine cytology, cystoscopy and the modern radiological techniques used in everyday clinical practice. We provide guidance on which patients should be referred for nephrological assessment and how to manage patients with negative investigations but ongoing significant haematuria.