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Collodion baby and harlequin fetus are two clinical entities that have characteristic appearances that mark their diagnosis. Yet, this is only the beginning of unraveling the congenital ichthyosiform dermatosis causing it, as they are mere phenotypic forms, not disease entities. Autosomal recessive ichthyosiform diseases are responsible for a majority of collodion babies. Both syndromic and nonsyndromic ichthyosis disorders cause collodion in babies; thus, these babies need to be thoroughly evaluated during hospital stay and during the follow-up period as well. The course of a collodion baby can range from self-healing to significant morbidity. It is more worse in the harlequin fetus. Neonates with both conditions need neonatal intensive care unit hospitalization. The collodion membrane gradually sheds in the majority, revealing the ichthyosis in time. Skin barrier maintenance is extremely important, as fragility of the skin leads to a sequelae of complications. Otherwise, treatment modalities depend on general care and temperature regulation, and retinoids have shown promising results. Both conditions are described, and the approach needed in evaluation of the collodion baby is highlighted.