The cardiac findings in eight patients, two of whom were female, with total lipodystrophy (Berardinelli‐Seip's disease) are reported. One of them had the acquired form of the disease. Four patients died at a mean age of 32 years. As far as we know, at least three of them most likely died for cardiac reasons, one shortly after recovering from an attempted suicide. All eight patients had hypertrophic hearts, mostly with deranged diastolic, but also systolic, function. One had pulmonary hypertension. We conclude that generalized lipodystrophy is a serious disease with cardiac affection leading to cardiac dysfunction and early death. There is no specific cardiac treatment, and the treatment should be according to the general guidelines for patients with hypertrophic, dysfunctioning hearts.