However, estimations have been generated using case reports, literature searches, and electronic medical record database searches, which indicate a prevalence of approximately 0.1–1 cases/million for CGL.4, 5, 6 The most severe form of lipodystrophy is CGL type 2 (CGL2), which is caused by mutations affecting BSCL2/seipin.7 Here, BSCL2 is linked to congenital generalized lipodystrophy type 2.