Elephantiasis nostras verrucosa (ENV) is a rare and dramatic sequela of chronic nonfilarial lymphedema. The condition is characterized by papules, verrucous lesions, enlargement, and woody fibrosis of the affected area. ENV is a progressive condition, and, without intervention, ongoing deformity and disability will result. Management of ENV is often challenging, but a variety of successful medical and surgical treatment strategies have been reported.