This study demonstrated that the CRISPR/Cas9-mediated HITI-based method can achieve precise and effective genome integration in the CYP4V2 gene both in BCD-iPSCs and in h-Cyp4v3mut/mut mice, and this treatment can cover almost 80% of BCD mutations. Here, CYP4V2 is linked to Bietti crystalline corneoretinal dystrophy.