Elephantiasis nostras verrucosa represents an infrequent clinical entity with cutaneous changes characterized by dermal fibrosis, hyperkeratotic verrucous and papillamotous lesions resulting from chronic non-filarial lymphedema secondary to infections, surgeries, tumor obstruction, radiation, congestive heart failure, and obesity. Although recurrent streptococcal lymphangitis is believed to play a critical role in the origin of elephantiasis nostras verrucosa, the exact pathogenesis of the disorder is not yet clear. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes but unfortunately there is no specific treatment for advanced cases. In this report, we present a patient who was treated by below knee amputation as a result of elephantiasis nostras verrucosa complicated with chronic tibial osteomyelitis.