Treatment of congenital missing of permanent tooth is often complicated by delayed diagnosis or inadequate treatment. The literature review was to focus on the etiology and diagnosis of congenital missing of permanent tooth, maintenance of primary tooth, and different options of treatment metods. The case report was to present a patient suffered from congenital missing of #13,#14,#15, #33,#45. We treated him with stainless steel crowns and composite resin filling for his carious primary teeth. We hope that protocol can provide clinicians a good reference for this kind of patients.