Congenital generalized lipodystrophy type 2, also known as Berardinelli‐Seip congenital lipodystrophy type 2 (BSCL2), is a disease with a wide spectrum of inherited and acquired syndromes characterized by lipodystrophy, hypertriglyceridemia, and insulin resistance.[45] Mutations of four genes have been linked to BSCL2, with BSCL2 mutations representing the most severe form of human BSCL2.[46] Our findings suggest that dysregulation of PRMT5 may also contribute to the development of BSCL2. Here, PRMT5 is linked to lipodystrophy.