Objective: Pheochromocytomas are a hallmark feature of von Hippel-Lindau disease (vHL). To our knowledge, this is the first systematic review with meta-analysis evaluating the frequency of pheochromocytomas and/or paragangliomas (PPGL) in patients with vHL, as well as among patients with different vHL subtypes. Design: Systematic review with meta-analysis. Methods: We searched on MEDLINE, Scopus and Web of Science. We included primary studies assessing participants with vHL and reporting on the frequency of PPGL. We performed random-effects meta-analysis to quantitatively assess the frequency of PPGL, followed by meta-regression and subgroup analysis. Risk of bias analysis was performed to assess primary studies’ methodological quality. Results: We included 80 primary studies. In 4263 patients with vHL, the pooled frequency of PPGL was 19.4% (95% confidence interval (CI)=15.9-23.6%, I²=86.1%). The frequency increased to 60.0% in patients with vHL type 2 (95%CI=53.4-66.3%, I²=54.6%) and was determined to be of 58.2% in patients with vHL type 2A (95%CI=49.7-66.3%, I²=36.2%), compared to 49.8% in vHL type 2B (95%CI=39.9-59.7%, I²=42.7%), and 84.1% in vHL type 2C (95%CI=75.1-93.1%, I²=0%). In meta-regression analysis, more recent studies were associated with a higher frequency of PPGL. All studies had at least one internal validity item classified as 'high risk of bias', with 13% studies having low risk of bias in all external validity items. Conclusions: PPGL are a common manifestation of vHL. Despite methodological limitations and differences across primary studies, our results point to the importance of PPGL screening in patients with vHL.