The clinical and biological features of the nine investigated family members carrying a PLIN1 variant recapitulated the FPLD4 cardinal signs (2) (i.e., lipoatrophy, muscular hypertrophy, facial acromegaloid features, insulin resistance-related ovarian dysfunction, and metabolic complications (e.g., hyperinsulinemia or insulin-resistant diabetes, hypertriglyceridemia, liver steatosis) (Table 1). This evidence concerns the gene PLIN1 and hypertriglyceridemia.