Sneddon-Wilkinson disease or subcorneal pustular dermatosis (SPD) is a rare, benign inflammatory skin disorder of unknown etiology. SPD is associated with various systemic disorders, including immunoglobinopathies and lymphoproliferative disorders. The relationship between SPD and immune dysfunction is unlikely to be incidental, although it still remains a mystery whether the associated gammopathies are primary or secondary to the pathogenesis of the disease. Herein, we report the first case of SPD in association with marginal zone lymphoma.