To our knowledge, this is the first reported case of digenic inheritance involving <i>LSS</i> and <i>TSPEAR</i>, which expands the clinical spectrum of <i>LSS</i>- and <i>TSPEAR</i>-associated disorders and supports consideration of broader genetic testing in children with congenital hypotrichosis and ectodermal features. This evidence concerns the gene TSPEAR and hypotrichosis.