Macrogyria/pachygyria falls on the wide spectrum of cortical developmental malformations, and its most severe form is known as lissencephaly (complete agyria), caused by variants in genes involving centrosome proteins (PAFAH1B1/LIS1), tubulins (TUBA1A, TUBB, TUBB2A, TUBB2B, TUBB3, TUBB4A, TUBG1, TUBGCP2, and TUBGCP6), microtubule motor proteins (DYNC1H1), actin-associated proteins (DCX), reelin (RELN and VLDLR), and forebrain development (ARX) (Mochida, 2009; Koenig et al., 2021; Kolbjer et al., 2021). This evidence concerns the gene ARX and Pachygyria.