Introduction: It is still unclear whether patients with intraductal papillary mucinous neoplasia (IPMN) harbor a higher risk of developing extrapancreatic malignancies (EPMs). Aim of this meta-analysis is to provide a pooled estimate of the incidence of EPM in patients with IPMN, with a particular focus on the comparison to the general population. Methods: Computerized bibliographic search on the main databases was performed through February 2021. Pooled effects were calculated using a random-effects model by means of DerSimonian and Laird test. Primary endpoint was the pooled incidence of EPM in IPMN patients. Additional outcome was the comparison of EPM occurrence between IPMN patients and general population, expressed in terms of standardized incidence ration (SIR) along with 95% confidence intervals (CIs). Results: Eighteen studies with 8709 patients were included. Pooled rate of EPM in patients with IPMN was 27.3% (95% CI: 20.5%-34.1%), with a pooled rate of 14.7% (9.4%-20.1%) of EPM detected previous to IPMN diagnosis and 6.6% (4.5%-8.7%) of synchronous EPM. Pooled rate of metachronous EPM was 10 (95% CI: 6-13)/1000 persons-year. No difference was observed according to IPMN histology and sex, whereas a significantly superior incidence of EPM was observed in patients with main-duct IPMN (36.7%, 25.4%-48% as compared to 26.2%, 17.6%-34.8% in branch-duct IPMN; P=0.03) and in Eastern studies (36.1%, 31.1%-41.1% versus 20.8%, 12.7%-28.9%; P=0.02). Pooled SIR of comparison with expected rates in general population was 1.01 (0.79-1.29); no difference was observed concerning rates of metachronous gastric cancer (SIR 1.60, 0.72-3.54) and colorectal cancer (SIR 1.29, 0.92-1.18), whereas biliary cancer was observed more frequently in IPMN patients (SIR 2.29, 1.07-4.93). EPM-related and IPMN-related mortality rates ere 6% (3%-9.1%) and 6.3% (4.2%-8.5%), respectively. Conclusion: Our meta-analysis demonstrates that IPMN patients harbor an overall rate of EPM as high as 27.3%, but rate of metachronous EPM is not superior to the general population.Figure 1.: Forest plot comparing observed/expected incidence of metachronous extrapancreatic malignancies in patients with intraductal papillary mucinous neoplasia. Standardized incidence ratio was 1.01 (0.79-1.29), with moderate evidence of heterogeneity (I2=22%).Table 1.: Analysis concerning gastric, colorectal, biliary cancer, and mortality.