The case report describes a 5-year-old boy with X-linked ichthyosis (XLI), significant seizures, short stature, and attention deficit hyperactivity disorder (ADHD). The skin showed an ichthyosiform appearance with diffuse scaling over the entire body. Seizures were manifested by deviation of the gaze upwards and weakness of all four limbs lasting approximately 2 minutes. Whole-exome sequencing (WES) revealed a pathogenic deletion of approximately 1.47 Mb at the Xp22.31 locus in a boy. The father had a wild-type allele, the mother a heterozygous deletion of approximately 1.14 Mb at the same locus. This variant was classified as pathogenic according to the American College of Medical Genetics and Genomics (ACMG) guidelines. XLI is a genetic skin disorder caused by defects in the steroid sulfatase (STS) gene, with 90% of patients having a deletion of this gene. The case highlights the complexity of XLI.