Like many solid organs, the kidneys are susceptible to a wide variety of systemic vascular diseases. Comprising a significant subset of these diseases are the vasculitides, broadly encompassing numerous inflammatory conditions of the blood vessels. However, many of these conditions are non-vasculitic and non-inflammatory, and differentiation of these entities is crucial to guide the initiation of proper therapy. These non-vasculitic diseases include coagulopathic conditions leading to vascular complications, hemolysis, and hematogenous processes that can affect multiple organ systems. These systemic diseases can result in both macrovascular and microvascular pathology, involving the arteries, veins, and smaller vessels, and management of these conditions can differ significantly depending upon the underlying pathophysiology. Because the clinical manifestations of these disease processes can be heterogeneous, ranging from renal dysfunction to life-threatening hemorrhage, proper recognition of these entities is essential to help guide clinicians to the correct diagnosis and prevent potentially disastrous complications. Many of these systemic vascular processes can be detected by non-invasive imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), and identification of their characteristic renal manifestations by radiologists is a critical component of patient care. This review covers a variety of these diseases and their imaging manifestations, to aid in their recognition and better equip radiologists to provide vital diagnostic information that can optimize patient care. Graphical abstract