Erythromelalgia is a rare poorly understood clinical condition characterized by intense burning pain, pronounced erythema, and increased skin temperature. Although there are many classifications of the disease, it can basically be divided into primary, which begins spontaneously at any age, and secondary, which is associated with myeloproliferative disorders-related thrombocythemia, polycythemia, collagen-vascular diseases, diabetes mellitus, peripheral neuropathy, autoimmune and infectious diseases, and use of certain medicaments. A wide variety of etiological conditions can cause erythromelalgia, all having a single common pathogenetic mechanism - microvascular arteriovenous shunting. The disease is characterized by severe pain associated with redness and hotness in extremities. The diagnosis is based on the medical history and clinical findings. The most useful oral medications for erythromelalgia seem to be aspirin, propranolol, clonazepam, cyproheptadine, drugs inhibiting serotonin re-uptake (venlafaxine and sertraline), tricyclic antidepressants (amitriptyline, imipramine), anticonvulsants (gabapentin), calcium antagonists (nifedipine, diltiazem), and prostaglandins (micoprostol). Erythromelalgia is usually chronic, sometimes progressive, and disabling disease, which can greatly affect the quality of life. Some patients have stable disease and get better, or even experience full resolution of the disease, with time. This review article presents the etiological basis, diagnostics, and therapy of erythromelalgia.