Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Pulmonary manifestations include hemorrhage, lung cavitary lesions, and pulmonary fibrosis. Within the kidney, GPA is known to cause rapidly progressive pauci-immune crescentic glomerulonephritis. Rare and severe cardiovascular manifestations include pericarditis, arrhythmias, myocarditis, and aortic valve disease. Our patient is a 43-year-old female with typical pulmonary and renal lesions from GPA and also acute myocarditis, multiple episodes of ventricular tachycardia, and a severe reactive thrombocytosis.