We describe bi-allelic variants in CAMSAP1, which encodes a molecule crucially important for minus-end microtubule stabilization, as a cause of a clinically recognizable, syndromic neuronal migration disorder with similarities to the “tubulinopathies.” Camsap1−/− mice displayed increased perinatal mortality, and proband-derived neural cell rosette lineages showed decreased cell proliferation and differentiation. Here, CAMSAP1 is linked to tubulinopathy.