Elephantiasis nostras verrucosa is a rare, chronic, deforming disorder characterized by hyperkeratosis and papillomatosis of the epidermis with underlying woody fibrosis of the dermis and subcutaneous tissue. Chronic lymphedema, either congenital or secondary to infection, surgery, radiation, neoplastic obstruction, obesity, portal hypertension, or chronic congestive heart failure, plays a pivotal role in the pathogenesis. Without appropriate intervention, the slowly progressive cutaneous changes will culminate in massive and grotesque enlargement of the affected body region. In the following case report, the natural history and the management of elephantiasis nostras are discussed.