IPS is caused by variants of the SLC2A4 gene encoding the fatty acid transporter protein 4 (FATP4) [5] and is inherited in an autosomal recessive manner. Although IPS has a favorable prognosis, neonatal complications can be fatal without implementing respiratory support in cases of respiratory distress, and recognizing this entity is thus vital for appropriate and planned management. The gene discussed is SLC2A4; the disease is ichthyosis prematurity syndrome.