Lipohyperplasia dolorosa (LiDo) is a disease that stands out due to its unique characteristics. It is characterised by the abnormal formation of subcutaneous fatty tissue in the extremities. The number and volume of adipocytes do not decrease under a low-calorie diet, physical exercise, or even after bariatric surgery. Pain, a significant and unique feature, is an additional obligate characteristic associated with the disease. What do we consider to be clinically typical? What seems to be unusual? The familial aspect of LiDo, with the frequent illness of other women in the affected person’s family, has recently led to an intensification of genetic studies. This aspect, which is of utmost importance, provides information about candidate genes that, as observed for some time in lymphoedema, can be decisive for the occurrence of the pathological picture in the event of a mutation.