Here we report 10 ichthyosis patients from three consanguineous and a nonconsanguineous Pakistani family, showing the typical phenotypes of ichthyosis including, scaling of the skin, erythema, keratoderma, hyper linearity of the palms and soles, and Pruritus, etc. The observed phenotypes were segregated with two homozygous splice site variants in TGM1 and SPINK5, a homozygous missense variant in SULT2B1, and a homozygous nonsense variant in FLG, respectively, identified through the whole exome sequencing (WES) approach. This evidence concerns the gene SPINK5 and ichthyosis.