Cutaneous polyarteritis nodosa is a rare disease that affects vessels of the deep skin and the subcutaneous tissue. Its etiopathology remains unknown. It predominantly affects skin, and the main cutaneous symptoms are subcutaneous nodules, livedo reticularis, and ulcerations that are mainly located on legs. Cutaneous polyarteritis nodosa can also cause extracutaneous symptoms (fever, malaise, myalgias, arthralgias, neuropathy). It is a chronic benign disease with a relapsing course. Diagnostic criteria for this disease were recently proposed and both clinical and typical histological features must be present to confirm the diagnosis. Treatment of cutaneous polyarteritis nodosa depends on the severity of the disease and the frequency of relapses. Mild forms limited to skin lesions should be treated with nonsteroidal anti-inflammatory drugs, colchicine and locally applied glucocorticosteroids. Cases that are refractory to the treatment, that recur with extracutaneous symptoms may require applying more aggressive approach (glucocorticosteroids orally, hydroxychloroquine, azathioprine, methotrexate, mycophenolate mofetil or intravenous immunoglobulins). The prognosis in cutaneous polyarteritis nodosa is favorable and the disease rarely turns into a systemic form.