Berardinelli-Seip congenital lipodystrophy is a rare genetic disorder characterized by the absence of body fat, leading to an abnormal distribution of fat in the body. Individuals with this condition typically have a normal or increased amount of fat in certain areas, such as the face and neck, while lacking fat in the limbs and trunk. This can result in a distinctive appearance and various health complications. The disorder is caused by mutations in specific genes, including AGPAT2 and BSCL2, which are involved in fat metabolism. People with Berardinelli-Seip congenital lipodystrophy often experience insulin resistance, diabetes, and other metabolic issues, requiring careful management of their health.