The cerebral cortex development is a process that involves the coordinated sequence of neural progenitor proliferation, neuronal differentiation and migration. Possible dysregulation of such processes results in malformations of cortical development (MCDs), such as periventricular heterotopias and polymicrogyria, which are characterized by morphological and functional cerebral complications. MCDs can be caused either by genetic or environmental factors. In this project, we selected to study a centriole and centriolar satellite gene, reported mutated in a patient with extensive polymicrogyria and heterotopia. By analyzing its expression profile during development, we observed species-specific differences which are suggestive of its plausible important role in human corticogenesis and malformations. Our preliminary data showed that primary’s cilium length was disrupted after the manipulation of the gene-target, which is in accordance with recent evidence that suggest the involvement of some MCDs’ causative genes with the organization and function of the centrosome-cilia axis. This leads us to currently investigate in animal models and in cerebral organoids (COs) the candidate gene’s manipulation impact on the developing cortex. At the same time, our goal is to use the CRISPR-Cas9 system to insert in human induced pluripotent stem cells the patient’s mutation and modelize it in COs. The analysis of our ongoing and future experiments aims at unmasking the cortical development and malformations relating mechanisms.