Collodion Baby is a descriptive term for newborns with the entire surface of the body wrapped in a rigid yellowish membrane that resembles solid and translucent parchment paper. A rare disorder that often occurs in preterm babies with low birth weight. Collodion babies can have defects in protein and fat metabolism due to genetic mutations. Management with emollients, fluid and electrolyte balance and overcoming infection. Case Report : Reported newborn came to the emergency room of Soeselo Hospital referral from Puskesmas with Collodion Baby and low birth weight. Yellowish skin is obtained throughout the body such as translucent thick parchment, fissures with some erosion and ecropions in both eyes. The patient is placed in an incubator with a temperature of 30-32o C, emollient therapy, topical antibiotics on fissures and erosions, prophylactic antibiotics and antibiotic creams for ectropion. Discussion : Collodion baby is a condition at risk of serious complications including: risk of infection, hypernatremic dehydration, fluid and electrolyte imbalances and temperature instability caused by epidermal lipid disorders and protein homeostasis, resulting in increased transepidermal water loss (TEWL) caused by mutations in the transglutaminase 1-TGM1, ALOXE3 or ALOX12B, ABCA12, HIPAL4/ichthyin, ABHD5 genes responsible for epidermal homeostasis. Conclusion: Collodion Baby is a disorder with severe corneal damage. The importance of proper and comprehensive management with the aim of minimizing complications can reduce morbidity and mortality.