We and others have described a severe and unusual form of lipodystrophy – pathological adipose tissue redistribution - caused by biallelic mutation of MFN2, encoding mitofusin 2 (Rocha 2017, Sawyer 2015, Capel 2018, Carr 2015, Pareyson 2015). The gene discussed is MFN2; the disease is lipodystrophy.