The pulmonary vasculitides are a rare group of heterogeneous disorders unified by the histopathologic finding of inflammation and destruction of the blood vessel wall. Diagnosis of these disorders is exceptionally challenging, given their highly variable clinical presentation, their relative rarity, and the overlap of the signs and symptoms of vasculitis with much more common entities. However, advances in the management of vasculitis allow for accurate diagnosis, risk stratification in the individual patient, and the implementation of evidence-based, effective pharmacologic therapies. This concise clinical review addresses the diagnosis and management of the patient with pulmonary vasculitis and provides an up-to-date review of the state of the field.