KIF7 is a kinesin motor protein that functions as a critical regulator of hedgehog (Hh) signaling through its localization to ciliary tips. KIF7 acts as both a negative and positive regulator of sonic and Indian hedgehog pathways 1, operating downstream of the SMO receptor through SUFU-dependent and independent mechanisms 2. At the ciliary tip, KIF7 organizes SUFU-GLI2 complexes, preventing inappropriate GLI2 activation in the absence of ligand while preventing GLI3 processing into its repressor form during pathway activation 3. This dual regulatory capacity is essential for proper developmental signaling in epidermal differentiation and chondrocyte development. KIF7 mutations cause multiple ciliopathic developmental disorders characterized by impaired hedgehog signaling. These include acrocallosal syndrome with postaxial polydactyly and corpus callosum abnormalities 4, Joubert syndrome presenting with distinctive hindbrain malformations and multi-systemic involvement 5, and supernumerary tooth formation through dysregulation of tooth number specification 6. Additionally, KIF7 variants have been associated with idiopathic scoliosis, potentially through altered keratin expression in intervertebral discs 7. Beyond developmental roles, KIF7 dysfunction contributes to cancer progression, particularly through aberrant hedgehog signaling in B-cell lymphoma and colon cancer, where KIF7 upregulation correlates with poor prognosis and increased tumor aggressiveness 8.