Primary cilia function as tumor suppressor organelles by controlling several signaling pathways involved in metabolic process, cell growth, proliferation and survival. In CCA, the loss of primary cilia, which is associated with HDAC6 overexpression, generates malignant cholangiocytes unable to properly regulate these signaling pathways. Therefore, ciliotherapies, which are based on ciliary restoration, might have a potential value for cancer prevention and treatment.