Cryofibrinogenemia is a rarely symptomatic disorder that is underrecognized due to the infrequency with which it causes symptoms. Although completely reversible, this disorder can be life threatening when untreated. In this review, the classification, pathophysiology, and clinical presentation of cryofibrinogenemia are described, based on case reports and prospective observational data. Diagnostic criteria are outlined, and therapies are assessed critically. This information should help clinicians in establishing a diagnosis of cryofibrinogenemia and initiating treatment.