Objective: Ichthyosis is a heterogeneous group of disorders characterized by hereditary keratinization of the skin. Ichthyosis means “fish skin.” There are at least 20 different types of ichthyosis. Among these, Harlequin-type ichthyosis is a rare, but often fatal, special form of congenital ichthyosis. Rates are higher in certain populations with higher probability of inbreeding. Babies are born with parchment-paper-like transparent membrane covering the whole body. Complications such as dehydration, prematurity, sepsis, electrolyte imbalance, and pneumonia could occur, adversely affecting survival. Case(s): We presented an ichthyosis case born from a dizygotic pregnancy of a Syrian woman living as a refugee in Turkey. Despite all the medical procedures performed in the neonatal intensive care unit, the baby who was diagnosed with Harlequin type ichthyosis died on the second postpartum day. Conclusion: Few cases of ichthyosis in twins have been reported. It is very important to benefit from prenatal screening and genetic counseling in the early diagnosis of such inherited, rare and fatal diseases.