BACKGROUND: Despite the increased use of total pancreatectomy with islet autotransplantation (TPIAT), systematic evidence of its outcomes remains limited. AIM: To evaluate the outcomes of TPIAT. METHODS: We searched PubMed, EMBASE, and Cochrane databases from inception through March 2019 for studies on TPIAT outcomes. Data were extracted and analyzed using comprehensive meta-analysis software. The random-effects model was used for all variables. Heterogeneity was assessed using the I measure and Cochrane Q-statistic. Publication bias was assessed using Egger's test. RESULTS: Twenty-one studies published between 1980 and 2017 examining 1011 patients were included. Eighteen studies were of adults, while three studied pediatric populations. Narcotic independence was achieved in 53.5% [95% Confidence Interval (CI): 45-62, < 0.05, I = 81%] of adults compared to 51.9% (95%CI: 17-85, < 0.05, I = 84%) of children. Insulin-independence post-procedure was achieved in 31.8% (95%CI: 26-38, < 0.05, I = 64%) of adults with considerable heterogeneity compared to 47.7% (95%CI: 20-77, < 0.05, I = 82%) in children. Glycated hemoglobin (HbA) 12 mo post-surgery was reported in four studies with a pooled value of 6.76% ( = 0.27). Neither stratification by age of the studied population nor meta-regression analysis considering both the study publication date and the islet-cell-equivalent/kg weight explained the marked heterogeneity between studies. CONCLUSION: These results indicate acceptable success for TPIAT. Future studies should evaluate the discussed measures before and after surgery for comparison.