Cold urticaria (CU) is characterized by the rapid onset of pruritus, erythema, and swelling after exposure to cold stimulus. CU can be classified into acquired and familial disorders. Acquired cold urticaria (ACU) is sometimes associated with serious underlying diseases, such as malignancies, systemic leukocytoclastic vasculitis and infectious diseases. A 55-year-old man was admitted to our hospital with 1-year history of urticarial rash, itching and angioedema on the parts of his body after cold exposure. He had no family history. Diagnosis was confirmed by applying ice cube test. Marginal zone B-cell lymphoma has not yet been reported as a cause of secondary ACU. Here, we report a patient with secondary ACU which was also diagnosed as marginal zone B-cell lymphoma.