Lipedema is a chronic, progressive disorder of adipose tissue, found almost exclusively in women, that involves disproportionate subcutaneous fat depositions, leading to progressive and symmetric enlargement of the legs. The condition usually appears at puberty or in the third decade of life. Many patients report a family history of such disorder. The diagnosis is usually based on the physical examination and medical history. If left untreated, lipedema may result in secondary lymphatic dysfunction, physical impairment and mental problems, leading to significant damage to patient's quality of life. The condition is frequently misdiagnosed as lymphedema or mistreated as obesity. Other than that, the differential diagnosis includes chronic venous insufficiency, constitutional variability of the legs, lipohypertrophy, Dercum's disease, Madelung's disease. The treatment options include diet and compression therapy, conservative treatment of secondary edema and surgical interventions. There is an urgent need for further studies on the pathogenesis of lipedema, epidemiology, diagnostic criteria and possible curative treatment.