We report the first case of ARCI resulting from a homozygous deletion of the 3’ end of intron 8, exon 9, intron 9, and part of exon 10 (831 bp) in the ALOX12B gene due to a mixUPD of maternal chromosome 17. The gene discussed is ALOX12B; the disease is autosomal recessive congenital ichthyosis.