Cold urticaria is a hypersensitivity to cold exposure (eg, wind, freezer compartments, cold water) resulting in histamine release. It usually presents as localized redness, burning, pruritus, and urticaria in the exposed areas, but may progress to a generalized systemic reaction, shock, and death. This condition may be familial or acquired. Familial cold urticaria is an autosomal dominant inflammatory disorder, manifested as a burning sensation of the skin occurring about 30 minutes after exposure to cold. Acquired cold urticaria may be associated with medication (eg, griseofulvin) or with infection. Cold urticaria may occur secondarily to cryoglobulinemia or as a complication of syphilis. Most cases of acquired cold urticaria are idiopathic. For diagnosis, an ice cube is usually applied to the skin of the forearm for 4–5 minutes, then removed, and the area is observed for 10 minutes. As the skin rewarms, an urticarial wheal appears at the site that may be accompanied by itching. Second-generation H1-antihistamines have been used as first-line treatment. In refractory cases, the addition of anti-immunoglobulin E (IgE) or cyclosporine may be considered.