Primary localised amyloidosis of the urethra is a rare entity with only 50 cases reported in the literature. The deposition of fibrillary proteins can lead to a range of symptoms. Importantly, the clinical and cystoscopic features may mimic urethral malignancy (haematuria, voiding difficulties, and a palpable urethral mass), and so thorough investigation is required in order to exclude malignancy, and to identify features of generalised amyloidosis which has a poor prognosis. Once diagnosed the prognosis of localised amyloidosis is excellent, although disease recurrence is possible. We describe a case of primary localised urethral amyloidosis presenting with visible haematuria and a urethral stricture and reviewed the literature regarding management of this rare condition.