Objective To perform occlusal reconstruction for patients with dentinogenesis imperfecta type Ⅱ (DGI-Ⅱ), observe the clinic effect and summarize the clinical experience. Methods The data of one family of DGI-Ⅱ were collected, and the clinical characteristics, treatment process and follow-up status of the proband (patient 1, female, 21 years old) and her relative (patient 2, male, 40 years) were retrospectively analysed. Results The main feature of these two cases of DGIⅡ was severe wear of the dentition, including upper and lower dentition defect in patient 2. After occlusal reconstruction and crown lengthening, patient 1 was restored by ceramic fixed partial denture to reconstruct the occlusion. Patient 2 was restored by removal partial denture after occlusal reconstruction. Patients were followed up 1 year after prosthetic treatment, and the subjective feelings of patients and clinical examinations indicated that the esthetics, mastication function and pronunciation of patients were significantly improved. Conclusion The principle of prosthetic treatment of patients with DGI-Ⅱ should be occlusal reconstruction of worn dentition. Occlusal reconstruction in combination with fixed or removable partial denture may yield favorable clinic effect.