Patients with chronic recurrent wheals are most commonly diagnosed with chronic spontaneous urticaria, although a number of autoimmune, autoinflammatory and malignant diseases can be suspected based on certain "red flags". These "red flags" are wheal duration of >24 hours, postinflammatory hyperpigmentation, systemic symptoms such as arthralgia and fever and/or elevated inflammatory markers. Herein, we detail a case of an adult patient initially diagnosed with CSU, discussing possible differential diagnoses and outlining options for treating the patient once a diagnosis has been established. We highlight a need for a careful examination of laboratory and histological findings and other investigations including serum immunofixation electrophoresis and genetic testing.