Background: Cold-induced urticaria can take place either due to direct cold exposure, cryoglobulinemia, or genetic component (such as cryopyrin-associated periodic syndrome), which leads to the rapid onset of urticaria and/or angioedema. It is more common in younger patients and more likely to affect females compared with males. Objective: To increase awareness of such systemic reactions of anaphylaxis and provide a focused review of the differential diagnosis, underlying mechanisms, broad workup, and management of this disease process for allergy/immunology fellows, residents, general physicians, and general practitioners. Methods: Pertinent information was included from the patient's clinical course. Also, a review of the available literature to include additional references that were obtained by using the works cited in the most up-to-date reviews was completed. Results: A case of a patient with cold-induced urticaria with common sequela was presented, followed by a discussion of the pathophysiology, diagnosis and its differential diagnosis, workup, and management. Conclusion: Cold-induced urticaria is a complex disease with several different catalysts. Providers should be aware of the different forms of cold-induced urticaria and recognize the risk for anaphylaxis in this patient population. Pearls and pitfalls of the diagnosis and management are provided.