Objective: To present our experience in managing neonate with Harlequin Ichthyosis (HI) by supportive treatment and administration of isotretinoin.
Case: A male infant was born at 35 weeks of gestation. He was born to a nonconsanguineous couple with no family history of any inherited skin disorders or previous children with skin disorders. He had an uncomplicated prenatal course. His birthweight, length, and head circumference was 2300 grams, 50 cm, and 32 cm respectively. 
After delivery, he was noted to have generalized edema with thick hyperkeratotic plates over his entire body and scalp and associated deep erythematous fissures (Figure 1). His eyelids and lips were everted showing ectropion and eclabium, respectively. The ears were small with closed pinna. His extremities were tight, edematous with contracted digits resulting a decreased capillary refill at distal ends. He had diffuse decreased range of motion, especially in the digits, wrists, knees and ankles secondary to skin tightness. 
The heart rate and respiratory rate were normal, the infant then transferred to the Neonatal Intensive Care Unit (NICU). The patient was consulted to dermatologists, ophthalmologists, orthopedist, otorhinolaryngologist. Initial management included broad-spectrum antibiotics (Amoxicillin-Clavulanate, 20mg/kg BID), enteral feeding, skin emollients, and eye ointment. Bathing of the infant was limited to once daily with warm normal saline ad high moisture soap. On day 10 of life, 1 mg/kg/d of isotretinoin was administered. Clinical improvement in the infant’s condition was seen over the subsequent weeks (Figure 2). 
Conclusion: Although HI is associated with poor survival in neonatal period, supportive care and treatment from an interdisciplinary team is required for effective management to improve survival rate.