Fibromuscular dysplasia is an uncommon angiopathy that occurs in young to middle-aged, predominately female individuals. The disease consists of a heterogeneous group of histologic changes, which ultimately lead to arterial narrowing. Clinical manifestations reflect the arterial bed involved, most commonly hypertension (renal) and stroke (carotid). Fibromuscular dysplasia is a pathologic diagnosis, but the characteristic changes seen on an angiogram can be used to make the diagnosis in the appropriate clinical setting. This noninflammatory disease is a common mimic of vasculitis. A very limited amount of new literature has been published in the past year about this relatively uncommon condition.