We present a curious case of localized cold urticaria restricted to the face in a 10-year-old girl. Testing for the condition using an ice cube was positive only in the facial area. After 2 years, the patient continues to experience localized urticaria only on her face on cold exposure. A review of the available published medical literature on cold urticaria was performed using Ovid and PubMed databases. The literature search was not limited to the English language. Only three other cases of cold urticaria localized to the face were identified. Upon review of the published reports on cold urticaria and discussion of classification and diagnostic testing, we conclude that cold urticaria clearly is a rare and poorly understood entity.