Familiar partial lipodystrophy (Dunnigan type) belongs to group of laminopathies. The main features of the disease include loss of subcutaneous adipose tissue from limbs and trunk, sparring the face and neck, associated with insulin resistant diabetes mellitus and dyslipidemia. Metabolic syndrome is responsible for atherosclerosis which predisposes to coronary heart disease in young people. Hormonal disorders, i.e. polycystic ovarian syndrome and acromegaloid features are found in some patients. Laboratory tests reveal insulin resistance, hyperinsulinemia, hyperglycemia, hypertriglyceridemia and low HDL-cholesterol. Mean FFA level is higher and leptin and adiponectin levels are lower in patients with lipodystrophy type Dunnigan in comparison to healthy people. Women predominance and intrafamilial variability are observed. The disease is characterized by autosomal dominant inheritance. The most frequent mutation is R482W in exon 8, but mutations in exon 11, affecting only lamin A, are also known. Treatment of dyslipidemia and diabetes (diet and pharmacotherapy) are main purposes of therapy.