Urticaria is a relatively common autoimmune/autoreactive skin disorder that may severely impair quality of life. Although rarely life-threatening, widespread urticaria and its associated angioedema can be an extremely disabling and difficult-to-treat condition. Patients may suffer symptoms such as pruritus and disfigurement due to wheals for years or decades. Urticaria is caused by cutaneous mast-cell degranulation attributed to immunological, non-immunological, and idiopathic causes. The last decade has seen some notable advances in the understanding of the etiology and pathogenesis of common forms of urticaria and their management. Furthermore, the wide diversity in urticaria subtypes has been identified and this reflects a partial understanding of the causes or factors that trigger it as well as the molecular and cellular mechanisms that are involved in its physiopathology. In addition, new instruments for diagnosing urticaria variants and for assessing quality of life in urticaria patients have been developed. Finally, several clinical trials have demonstrated the efficacy of novel treatment approaches for urticaria, while other therapeutic concepts are under development. The objective of this article was to review the literature to be able to offer the readers comprehensive and updated information on the basic etiological and physiopathological mechanisms and to make special emphasis on the current management of urticaria, thus promoting continuous medical education.