Genetic and acquired lipodystrophies are disorders of adipose tissue distribution. In this review we will emphasize its phenotype, metabolic and genetic particularities. Special stress will be given to the most prevalent lipodystrophy ‑that associated with HIV infection - describing the similarities with congenital lipodystrophies and its pathogenic mechanisms. It will be discussed the pathways involved in development, differentiation and death of adipocytes, emphasizing that both genetic and acquired, including that associated with HIV can induce changes in adipogenesis and how the two main adipose tissue dysfunctions - obesity and lipodystrophy - can cause a similar metabolic profile (diabetes, hypertension, dyslipidemia) with increased cardiovascular risk. Rare monogenic laminopathies and other genetic lipodystrophies could be relevant to understand common pathways - physiopathology of aging, atherosclerosis process, vascular endothelial cell dysfunction, frailty syndrome and common disorders such as type 2 diabetes, insulin resistance, dyslipidemia, hypertension and metabolic syndrome. Also, some lipodystrophies can be associated with premature aging and precocious cardiovascular disease. The authors aimed to give a focus on the particularities of each of the issues addressed.