Among lipodystrophies, the Barraquer-Simons syndrome or "parital lipodystrophy of the cephalothoracic type" is a rare condition of unknown origin characterized by a progressive atrophy of the subcutaneous fat of the upper part of the body. We report a 34-year-old woman with progressive loss of subcutaneous fat during 20 years, first affecting the face and spreading to the upper part of the body, equally presenting hypertrophy of the lower body fatty tissue. An overview of this rare syndrome is made.