Amyloidosis is a heterogeneous group of disorders characterised by extracellular deposition of amorphous proteinaceous material in various tissues. Primary bladder amyloidosis is a rare disease and its presentation is of clinical interest for the urologist because it resembles that of a malignant bladder tumour. Transurethral resection and histological examination is essential to exclude malignancy and to demonstrate the presence of amyloidosis. It is also important to exclude systemic amyloidosis. A new case is presented of primary bladder amyloidosis, briefly discussing its clinical, treatment, and monitoring.