The trigeminal autonomic cephalalgias (TACs) are relatively rare, but they represent a distinct set of syndromes that are important to recognize. Despite their unique features, TACs often go undiagnosed or misdiagnosed for several years, leading to unnecessary pain and suffering. A significant proportion of TAC presentations may have secondary causes. The underlying pathophysiology of TACs is likely rooted in hypothalamic dysfunction and derangements in the interplay of circuitry involving trigeminovascular, trigeminocervical, trigeminoautonomic, circadian, and nociceptive systems. Recent therapeutic advancements include a better understanding of how to use older therapies more effectively and the identification of new approaches. TAC syndromes are rare but important to recognize because of their debilitating nature and greater likelihood for having potentially serious underlying causes. Although treatment options have remained somewhat limited, scientific inquiry is continually advancing our understanding of these syndromes and how best to manage them.