Lipedema is a chronic and progressive disease that mostly affects women and appears to be a clinical entity thought to be related to both genetic factors and fat distribution. It usually affects women starting most often between puberty and the third decade of life. There is an abnormal deposition of subcutaneous adipose tissue that leads to a bilateral disproportionate increase in volume of lower extremities and sometimes arms as well typically sparing hands, feet and trunk. There is possible inheritance of X-linked dominant or autosomal dominant pattern with sex limitation involved in this condition. Lipoedema is symmetrically distributed on both sides and is often associated with pain, oedema, and easy bruising. It can lead to daily functioning impairment,  considerable disability and psychosocial distress. Lipoedema is often misdiagnosed as lymphedema or simple obesity  because of unfamiliarity of clinicians with this condition. There should be a proper approach adopted by health care professionals to be familiar with this disease and prevent advanced cases of lipoedema that can lead to venous disease (veno lipoedema), lymphatic abnormalities (lympho-lipoedema), and subsequently ulceration and recurrent infections. We will further discuss the epidemiology, pathophysiology, clinical presentation, types and stages of lipoedema  assessment and diagnosis and management options available for this often misdiagnosed disease.