The purpose of this case-based review is to highlight cranial nerve involvement in granulomatosis with polyangiitis (Wegener's). In this disease, cranial nerve involvement may be less frequent than other neurological manifestations, but often goes unrecognized by physicians as a sign of the disease, and its prevalence and importance is likely underestimated. Awareness of this aspect of the disease is necessary to make the proper diagnosis rapidly, as it can be a major feature of a patient's presentation. We also briefly discuss the known pathogenic mechanisms, which could be important when selecting the best therapeutic option.