Omenn syndrome: the drama of a family, congenital ichthyosis is not always mundane!

Background: The case we are reporting is about one of the rare manifestations severe combined immunodeficiency, Omenn syndrome (OS). Case presentation: A 43-days-old female presented with thick diffuse erythrodermic scaly ichthyosiform lesions on scalp, face, and trunk since birth. lymphadenopathy, splenomegaly, growth retardation as well eosinophilia increased serum IgE levels. pregnancy was planned for an allograft bone marrow, but procedure not carried out due to a persistent post-covid pneumopathy bilateral parenchymal condensation that resulted in death. Conclusion: This report intends incite clinicians be alert this possible diagnosis underrate immune deficiency neonatal erythroderma.