Water is ubiquitous in daily lives and generally regarded as harmless, yet for some patients, water is a source of great discomfort. Aquagenic Urticaria (AU) is a form of physical urticaria that occurs with cutaneous exposure to water, including sweat and tears. Patients present with characteristic 1–3 mm folliculocentric wheals with surrounding 1–3 cm erythematous flares within 20–30 minutes following skin contact with water. Aquagenic urticaria most commonly develops on the trunk and upper limbs. It is associated with pruritus and an uncomfortable prickling or burning sensation. With around 100 cases, the disease has received little to no attention from the scientific community. At this point, the pathophysiology of AU is not well known, however, it seems to be mediated both independently and in dependence on histamine. The water challenge test and a comprehensive clinical history are used to make the diagnosis. To rule out the possibility of other physical urticarias, certain patients might require additional testing. This paper focuses on the pathophysiology, diagnosis, and management of aquagenic urticaria. By raising awareness of this condition among health care providers, it may be possible to identify a larger group of patients to assist in further study.