Trichohepatoenteric syndrome (THES), also known as syndromic diarrhea, is a rare genetic disorder inherited in an autosomal recessive pattern due to mutations in the Ski2-like ribonucleic acid (RNA) helicase (SKIV2L) gene, also known as superkiller complex 2 (SKIC2) (OMIM # 614602) and the tetratricopeptide repeat domain 37 (TTC37) (SKIC3) (OMIM # 222470) genes [1,2]. This evidence concerns the gene SKIC3 and trichohepatoenteric syndrome.