Elephantiasis nostras verrucosa represents an uncommon yet distinct clinical entity resulting from chronic lymphedema of an extremity or body region. Characterized by profound non-pitting edema with cobblestone-like papules, plaques, and nodules, it typically occurs secondary to infections, surgeries, tumor obstruction, radiation, congestive heart failure, and obesity. This progressively deformative disorder has been treated with various medical and surgical measures. In the following case report, the history, clinical, and pathologic appearance of elephantiasis nostras verrucosa are discussed, as well as newer treatment options.