Seventeen cases of collodion baby are reported. Clinical aspects, complications, treatment, final outcome and family history were studied. We did not observe any clinical features in the collodion baby that could serve as a clue in predicting the final diagnosis. Infections were observed in nine, hypothermia in five and hypernatraemic dehydration in four cases. Skin infection mainly occurred in babies treated with emollients (petrolatum, lanolin and cetomacrogolis cream were used). We therefore recommend treating the collodion baby in a humidified incubator, if necessary with intravenous rehydration, but not to use emollients. The final outcome of these study patients was erythrodermic autosomal recessive lamellar ichthyosis in seven cases (41%), non-erythrodermic autosomal recessive lamellar ichthyosis in three cases (18%), Sjögren–Larsson in one case (6%), epidermolytic hyperkeratosis in one case (6%), acute neonatal variant of Gaucher disease in one case (6%) and normal skin in four cases (24%).