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The term lipedema is misleading, although it is true that there is an alteration of the fatty tissue, there is little evidence regarding edema. It is considered a disease since the last ICD‑11 (2019), included in the section of pathologies of skin, fat and subcutaneous cellular tissue. However, there are still doubts about whether it should be considered a disease or whether it is an aesthetic disorder. The diagnosis of lipedema can present challenges, since it is often confused with other nosological entities; especially with lymphedema. The aim of this task is to determine the basis for a correct differential diagnosis that helps to recognize lipedema as an entity with its own characteristics in order to facilitate its early identification. Clinical manifestations, plus the anamnesis and detailed examination of each patient, may be sufficient to reach a correct diagnosis. In case of doubt, there are tests capable of differentiating between lipedema and lymphedema. Differential diagnosis between lipedema and lymphedema should be made thoroughly and early in order to offer early advice and specific treatment to patients.