Giant cell arteritis (GCA) is a granulomatous vasculitic disorder that affects cranial arteries, as well as large arteries, including the aorta. The possibility of GCA should always be considered in headache developing in elderly people. Temporal artery biopsy is usually required to establish the diagnosis. Prompt institution of steroid therapy reduces the risk of complications, such as visual loss and major ischaemic events. Primary central nervous system vasculitis (PCNSV), or primary angiitis of the central nervous system (PACNS), causes headache and neurological symptoms. Patients affected by PCNSV can exhibit thunderclap headache. In such cases, scrutiny for subarachnoid haemorrhage, pituitary apoplexy, and venous thrombosis is warranted. Differential diagnosis between PCNSV and reversible cerebral vasoconstriction syndrome can be challenging. However, this differentiation is important, because they require distinct therapeutic strategies. Steroid and cyclophosphamide are used to treat PCNSV.