KIF26A is an atypical kinesin that functions as a non-motor microtubule-binding protein critical for nervous system development 1. Unlike canonical kinesins, KIF26A lacks ATP-dependent motor activity but serves as a microtubule stabilizer regulating neurite growth and neuronal migration 2. In the enteric nervous system, KIF26A represses GDNF-Ret signaling by inhibiting GRB2-dependent pathways, essential for proper enteric neuron migration and differentiation 3. In cerebral cortex development, KIF26A is preferentially expressed in excitatory neurons during early gestation, where it regulates radial migration, axonal/dendritic growth, and apoptosis through modulation of MAPK, MYC, and E2F signaling pathways 1. Biallelic loss-of-function KIF26A variants cause severe congenital malformations including polymicrogyria, cortical dysplasia, heterotopia, and congenital hydrocephalus 4, often accompanied by megacolon and intestinal pseudo-obstruction with aganglionosis 5. KIF26A variants also associate with congenital cranial dysinnervation disorders affecting eye movements 6. Additionally, reduced KIF26A expression in gastric cancer suppresses metastasis by inhibiting focal-adhesion kinase signaling and epithelial-to-mesenchymal transition, though this appears unrelated to developmental functions 7. Clinical severity correlates with variant type—missense variants cause protein destabilization, while truncating variants produce complete loss of function 5.