THOC6 and THOC6-related developmental delay-microcephaly-facial dysmorphism syndrome: Beaulieu–Boycott–Innes syndrome (BBIS) is a rare autosomal recessive neurodevelopmental condition linked to the THO complex subunit 6 (THOC6).[1] As of early 2020, 7 years after the syndrome was first described and its molecular basis identified, only 19 affected individuals have been reported in the literature.[2] THOC6 is an integral component of the THO/transcription/export (TREX) complex, which plays a crucial role in messenger ribonucleic acid (mRNA) transcription, processing, and the export of spliced mRNA.[3]