Localized amyloidosis of the penile urethra and corpus spongiosum is rare. The pathogenesis is obscure and optimal management of such patients has not been defined. We report a case that was treated with transurethral removal of amyloid tissue, which was obstructing the urethral outlet. Previously, the patient had been treated with dilation with temporary relief of the symptoms. He was disease-free 1 1/2 years postoperatively.