Elephnatiasis Nostras Verrucosa (ENV) is a rare clinical condition, a progressively disfiguring disorder characterized by verrucous, nodules and cobblestone-like papules associated with non-pitting edema of the lower extremities. Histopathology is characterized by epidermal hyperkeratosis and dermal fibrosis. We report the history of a 32-year-old man who presented with 5 years history of right lower limb edema covered by nodular and papular skin lesions. Proper investigations put the diagnosis of ENV. The patient was successfully treated by debridement followed by skin graft using the technique of dermal regeneration. Management of ENV remains challenging. In the absence of randomized controlled trials on the best modalities of treatment, it is important to report such rare cases and their treatment outcome.