Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis) is a type of small artery necrotizing vasculitis that presents with various organ manifestations and disease severity. The most commonly and severely affected organs include the nasopharynx, lungs, and kidneys. However, it can have atypical presentations and lead to misdiagnosis. Here we present a case report of a patient diagnosed with GPA complicated by diffuse alveolar hemorrhage (DAH), splenic infarctions, and stroke.