Introduction: Amelogenesis imperfecta (AI) is a rare genetic disease affecting both dentitions. Factors such as age, socioeconomic status, dentition and AI type and severity should be taken into consideration in treatment planning. Aim: This retrospective study aimed to assess the survival rate of AI main restorative options and the effects of gender and dentition type. Methods: The study sample comprised 28 dental records of patients aged 5-17 years affected by AI (15 females, 13 males) and with anterior and/or posterior restoration in primary and/or permanent tooth/teeth. The fate of each restoration was classified into three categories: failed, withdrawn and censored and analyzed by the life table method of survival analysis. Results: Out of 233 restorations performed, the most frequently used restoration was preformed metal crowns (PMCs), followed by anterior composite, posterior composite, adhesive casting, Ketac fill and amalgam respectively. The two main restorations, PMCs and anterior composite were included in the statistical analysis. The survival rate of PMCs was significantly higher than anterior composite (p<0.001). The anterior composite restorations survived significantly longer in males (p<0.05). Females had significantly better survival rate of PMCs (p<0.05). There was no statistically significant effect of the operator group of restoration survival. Conclusion: The anterior composite restorations survived significantly longer in males and females had significantly better survival rate of PMCs than males.