The article describes the case of a child with hypotrichosis type 14 (HYPT14, OMIM: 618275) complicated by ectodermal dysplasia type 14 (ED14, OMIM: 618180). The child had compound heterozygous variants in the LSS gene: c.1025T>G; p.(Ile342Ser) and c.3G>A (inherited from the mother) and a heterozygous variant in the TSPEAR gene: c.872G>A; p.(Arg291Gln). All variants were classified as variants of uncertain significance (VUS) according to ACMG criteria. The patient's phenotype overlapped the HYPT14 and ED14 traits. Immunomodulatory treatment with a JAK inhibitor produced only a transient response without permanent hair growth. This is the first reported case of digenic inheritance involving LSS and TSPEAR, expanding the clinical spectrum of these disorders. The findings support wider genetic testing in children with congenital hypotrichosis and ectodermal features.