Trichothiodystrophy (TTD) is a form of syndromic ichthyosis, an autosomal recessive disorder caused by variants in genes encoding subunits of the transcription/repair factor IIH (TFIIH), a multiplex protein that is essential for nucleotide excision repair (NER) and RNA polymerase II-driven transcription [4]. The gene discussed is ERCC2; the disease is ichthyosis.