A 24 year old uncircumcised man presented with a 1 month history of a painful, fungating lesion on his glans penis. Following biopsy, and further clinical developments, a diagnosis of Wegener's Granulomatosis (WG) was made. The penile lesion was treated with surgical debridement, and a penile stump was salvageable. This is only the fourth case of WG initially presenting with a penile lesion to be reported in the literature.