Graves' disease is an autoimmune disorder in which TSH receptor antibodies play the main pathogenic role. Besides the usual clinical presentation as hyperthyroidism, extrathyroidal manifestations can develop. Thyroid dermopathy is one of them and it is present in about 0.5-4.3% of cases, mostly in patients with thyroid orbitopathy as main extrathyroidal manifestation. Dermopathy can manifest in different forms: non-pitting edema, plaques, nodules and elephantiasis. We are reporting a case of a patient with thyrotoxicosis caused by Graves' disease with ocular extrathyroidal manifestation and dermopathy in elephantiasis form, confirmed by punch biopsy. Corticosteroid therapy significantly improved ocular manifestations, as well as skin lesions. Elephantiasis represents the most severe form of this disease and can be resistant to any treatment thus why it is a major therapeutic challenge.