Amyloidosis refers to a non-neoplastic heterogeneous group of disorders related to the extracellular deposition of insoluble fibrils in different organs. 1 Amyloidosis can be primary, secondary, and hereditary. Primary amyloidosis (AL) is usually related to plasma cell abnormalities. On the contrary, secondary amyloidosis (AA) occurs due to longstanding infections, inflammation, or neoplastic insults. Eventually, hereditary amyloidosis (ATTR) is related to autosomal-dominant inherited mutations in transthyretin protein gene. 1 Amyloidosis can be systemic, which is usually progressive and fatal or localized. Localized amyloidosis of the urinary bladder is rare, with the most extensive series reported 31 cases. 2 The clinical importance of bladder amyloidosis is that it mimics bladder cancer. Although amyloidosis is a benign condition, it was associated with urothelial carcinoma in 48% of 21 cases reported. 3 Here, we present a case of localized bladder amyloidosis with a review of the literature. Interestingly, this is the first case