Using a variety of approaches, we show that (i) Rpgrip1l is required in ciliated cells surrounding ventricular cavities for maintaining a straight axis; (ii) at juvenile stages, rpgrip1l mutants develop cilia defects associated with ventricular dilations and loss of the RF; (iii) increased urp1/2 expression in rpgrip1l mutants does not account for spine curvature defects; and (iv) a pervasive astrogliosis process associated with neuroinflammation participates in scoliosis onset and progression. Here, FERMT1 is linked to scoliosis.