Macular corneal dystrophy(MCD)refers to corneal stromal dystrophy, which can cause progressive visual field defects. MCD belongs to autosomal recessive disease. Mutation of CHST 6 gene is considered to be a pathogenic factor. In general, full-thickness penetrating keratoplasty has become a standard treatment for improving vision. Now more advanced surgical interventions such as deep anterior lamellar keratoplasty(DALK)and phototherapeutic keratectomy(PTK)have also been shown to be important in the treatment of MCD. Some new technologies, such as gene targeting therapy and enzyme replacement therapy, are also being studied as potential permanent solutions for MCD. The pathogenesis, genetic basis, clinical manifestations, treatment and prognosis of this disease are reviewed in this paper.