Granulomatosis with polyangiitis (GPA) is a relatively rare systemic autoimmune disorder of small and medium size blood vessels affecting multiple organs with a wide range of clinical presentations. We present a 57-year-old Caucasian male who presented to the ER with midsternal chest pain. He was hospitalized for non-ST elevated myocardial infarction (NSTEMI) and later diagnosed with pauci-immune necrotizing crescentic glomerulonephritis confirmed with renal biopsy.