Introduction: Lipedema is a chronic feminine disease that causes abnormal fat deposition in lower limbs and occasionally upper limbs. Easy bruising and pain are common. Lipedema patients suffer from both physical and psychological disability. Despite the relatively high prevalence and the impact on the quality of life, little is known about the disease. Most patients are misdiagnosed as lymphedema or obesity. The aim was to perform a non-systematic review on lipedema literature, related to diagnosis and therapy. Evidence acquisition: A literature search was performed by three researchers, to retrieve pertinent articles in PubMed 5 Web of Science 6 and ResearchGate 7 from the last 20 years. The documents were selected from systematic reviews, reviews, meta-analyses, consensus documents and guidelines, randomized controlled trials and retrospective, cohort and clinical series. Evidence synthesis: Regarding pathophysiology, alterations are not exclusively confined to adipose tissue. Lymphatic dysfunction, cardiovascular variations, blood capillary fragility and increased permeability are common. Patients often present with neurological alterations and hormonal malfunction. Elevated cutaneous sodium has been documented. Due to the absence of a defined etiology, evidence-based research is difficult in the management of lipedema. Conclusions: The setting of realistic expectations is important for both patient and medical care provider. The major aims of the management are multimodal for improvements in the quality of life; reduction in heaviness and pain, reshaping the affected limbs, weight control, improvements in mobility. Compression garments, physical therapy, exercise regimens, diet and psychological counseling are necessities. For selected patients, surgical treatment is an added option.