Berardinelli's Syndrome or Laurence Seip Syndrome is a rare inherited disorder presenting in early childhood. Glucose and fat metabolism are mainly deranged with clinical presentation of generalized lipoatrophy, hypertriglyceridemia and insulin resistance in early childhood. There is no curative treatment, anticipation and symptomatic treatment of complications is the reported management. Precise metabolic control was challenging during anesthesia for a seven year old child with multisystem involvement like an aged patient.