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Lipedema is a clinical entity frequently misdiagnosed or confounded with primary lymphedema. Lipedema is an adipose tissue disorder, also pathological itself, that occurs almost exclusively in obese women. It is characterized by bilateral enlargement from hip to ankle due to abnormal deposits of subcutaneous fat, usually sparing the feet. This disease usually occurs at or just after puberty. Patients may complain of pain, easy bruising of the affected areas with moderate to severe sensitivity to digital pressure or pinching, and mild edema after orthostatism. Lipedema results in considerable frustration and distress resulting from the cosmetic appearance. Imaging studies using computed tomography, magnetic resonance imaging, and lymphoscintigraphy are not indicated, except if the diagnosis is atypical or doubtful or to confirm lipedema. Long-term evolution may alter lymphatic system and lead to a lipo-lymphedema, then involving the foot, with specific complications such as cellulitis. Lipedema management is not codified and includes weight loss (which poorly improves leg appearance or discomfort), psychological counselling, compression therapy often poorly tolerated and physical activity, particularly aquatic. Liposuction using tumescent local anesthesia, may reduce edema, spontaneous pain, sensitivity to pressure, bruising and may improve appearance resulting in a notable improvement of quality of life. Clinical and physiopathological studies are still required to enable improved management of women having lipedema.