Granulomatous diseases have a varied etiology that includes autoimmune, infectious, idiopathic, and hereditary causes. The unifying factor in these diseases is the formation of granulomas, which histologically are mononuclear inflammatory cells or macrophages surrounded by lymphocytes. Granulomatous diseases often have systemic manifestations that affect organs throughout the body. Granulomatous diseases with head and neck manifestations include granulomatosis with polyangiitis, Churg-Strauss syndrome, Behçet disease, chronic granulomatous disease, and sarcoidosis. Infectious causes include tuberculosis, cat-scratch disease, syphilis, leprosy, actinomycosis, rhinoscleroma, and fungal infections. In the head and neck, granulomatous disease may affect the orbits, sinonasal cavities, salivary glands, aerodigestive tract, temporal bone, or skull base. Imaging findings include sinonasal opacification, ocular and other soft-tissue masses, osseous erosion, airway narrowing, lymphadenopathy, and salivary gland infiltration. Vascular involvement may also be evident, with displacement, narrowing, or occlusion of arteries and veins. Some radiologic findings of granulomatous processes have a considerable overlap with findings of malignancy, and a radiologic differential diagnosis inclusive of both is critical to avoid incorrect clinical treatment. Without the benefit of a prior clinical diagnosis, laboratory findings, or suggestive clinical signs and symptoms, granulomatous diseases may be difficult to differentiate radiologically. Although individual granulomatous diseases may have overlapping findings at imaging, certain radiologic findings should prompt the inclusion of granulomatous diseases in the differential diagnosis, thus facilitating appropriate clinical management.