The exposure to cold can induce the development of wheals and angioedema in a group of susceptible individuals: this phenomenon is largely known as cold-induced urticaria. The pathogenesis of cold-induced urticaria is not yet understood, although both autoallergens and immunoglobulin (Ig)E-mediated autoimmunity are presumed to be involved. Flares of cold-induced urticaria might depend on the release of histamine and other pro-inflammatory mediators, such as Interleukin (IL)-1, which is the predominating stakeholder of cryopyrin-associated periodic syndrome, a genetic disease characterized by cold-induced skin manifestations, including urticaria-like rashes. The majority of occurrence of cold-induced urticaria in children is idiopathic, but forms secondary to systemic conditions have been also reported. Primarily, the diagnosis remains a clinical process based on the history of patient, cold stimulation tests, and a few laboratory results, which could be useful for the excluding any underlying disorders. The general rules to manage cold-induced urticaria in children can be summarized with cold avoidance, treatment with nonsedating antihistamines, and the anti-IgE monoclonal antibody omalizumab in selected patients. Familiar forms of cryopyrin-associated periodic syndrome could be prevented even in pediatric patients from the selective IL-1 blockade. Injectable epinephrine must be immediately used to manage the potential life-threatening manifestations occurring in a minority of children with cold-induced urticaria.