Erythromelalgia is a very rare disease that can cause intermittent painful redness, heat and oedema affecting more commonly the lower extremities, mostly aggravated by heat and dependence, and can lead to significant disabilities. Whereas primary erythromelalgia is thought to be caused by familial or sporadic mutation in the SCN9A gene which encodes for the voltage-gated sodium channel, secondary erythromelalgia can be caused by different conditions including myeloproliferative, blood and autoimmune disorders. Differential diagnoses include peripheral neuropathy, complex regional pain syndrome, dermatitis, skin infections, autoimmune disease skin involvement, osteomyelitis, arterial or venous insufficiency and Fabry disease. Management for erythromelalgia can be very difficult and challenging and should have a multidisciplinary approach. There is no single effective treatment for erythromelalgia. Management includes patient education, controlling secondary and underlying factors, cooling techniques and selective medications such as aspirin in patients with myeloproliferative disorders.