Collodion baby refers to a newborn with collodion membrane covering the whole body. Most of them evolve into a form of autosomal recessive congenital ichthyosis(ARCI), but about 10% of them heal spontaneously. This mild phenotype is known as self-healing collodion baby (SHCB). To date, ALOX12B, ALOXE3, TGM1 and CYP4F22 gene mutations have been implicated in the etiology of SHCB. A boy was born at 38 weeks gestation from healthy parents. At birth, he showed collodion membrane that covered the whole body. A skin biopsy from the thigh showed pronounced hyperkeratosis with parakeratosis, but the stratum granulosum was normally present. Transmission electron microscopy findings revealed numerous lipid droplets in the thickened horny layer and the presence of unprocessed lamellar body contents in the intercellular space of the stratum corneum. Whole-exome sequencing identified a heterozygous missense mutation in ABCA12 gene. At a follow-up visit at 4 months of life, he was completely healed showing normal appearing skin. Although we could not find any homozygous or compound heterozygous mutation in the known ARCI-related genes, the patient was diagnosed as SHCB according to the characteristic clinical progression. In previous studies, there have been SHCB cases who did not show any mutations in the ARCI-related genes. Here, we report a rare case of SHCB and suggest a possibility that other unidentified genes may be involved in this case.