Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA) associated; vasculitis affecting small vessels and mainly cause upper and lower respiratory tract and renal involvement. Urogenital involvement is extremely rare and poorly described in the literature. We report herein a case of a 46-year old immunocompetent patient presenting with recurrent urinary tract infections and bladder retention with incidental findings of sinusitis, and pulmonary mass revealing GPA.