Granulomatosis with polyangiitis (GPA) commonly presents with glomerulonephritis and inflammation of upper and lower respiratory tracts. It can also involve other organs including those of the urinary tract. The involvement of the urethra is very rarely reported. We present a case of GPA in a patient who had recurrent urinary tract infections and an acute bladder outlet obstruction due to a urethral thickening by GPA. In this report, we discuss urethral involvement with GPA. The incidence of such involvement, as with other urinary tract organs, might be underestimated. It can affect both sexes, with male predominance, and can occur at any age. It responds to standard GPA medical treatment but may require surgical intervention. Rheumatologists should be aware of this limited form of GPA as early recognition and treatment can decrease the risk of complications.