Lipodystrophic syndromes represent a heterogeneous group of very rare diseases inherited or acquired, characterized by altered body fat amount and/or repartition and metabolic alterations including marked insulin resistance and dyslipidemias. We report the case of severe hypertriglyceridemia at 96 g/L in 28-year-old Moroccan women admitted for decompensated diabetes mellitus and generalized lipodystrophy. This case illustrates the value of hypertriglyceridemia in the diagnosis and monitoring of lipodystrophic syndromes whose diagnosis is made by clinical assessment and laboratory findings.