Centrosomes are microtubule organizing centers that include a pair of centrioles, one of which functions as a basal body serving as a template for cilia formation. Abnormalities in centriole biogenesis, centrosome structure/function, or formation and maintenance of cilia result in a heterogeneous group of human diseases, including central nervous system disorders, cancer and ciliopathies. We and others have recently described the human ciliopathies oral-facial digital syndrome type VI and Joubert Syndrome due to loss-of-function mutations in KIAA0753, a gene involved in centriole duplication. Current knowledge on how these mutations in KIAA0753 affect basal body function and ciliogenesis is limited. In this study, we generated two kiaa0753 null mutants in zebrafish using the CRISPR/Cas9 system. We observed that the Kiaa0753 protein is critical in zebrafish survival because mutants died between days 5 to 15. We demonstrated that the kiaa0753 null mutants exhibited a phenotype associated with cilia defects, including a curved body, edema, retinal abnormalities, renal cysts, and lack of a swim bladder. Further analysis of the neural masts revealed that the mutant fish had shorter and deformed cilia. Cultured cells from mutant embryos showed defects in cell proliferation, supporting the importance of kiaa0753 in cell division. Our novel zebrafish model shows the importance of kiaa0753 in embryonic development, specifically in ciliogenesis, and can be a useful tool for understanding the biology of centrosomes.