Context IDH1 is a pheochromocytoma/paraganglioma (PPGL) susceptibility gene, though its role, especially in the Chinese population, has not been characterized. Objective To determine the prevalence of somatic IDH1 hotspot variants in a large cohort of Chinese patients with PPGLs and to summarize associated phenotypes. Design Retrospective cross-sectional study. Setting Multiple tertiary-care centers in China. Participants This study was based on a main cohort of 1141 patients with PPGLs from two centers. We included 50 cases with urinary bladder paragangliomas (UBPGLs), from which 29 were part of the main cohort and 21 from other centers. Two additional cases with IDH1 hotspot variants not part of the main cohort were also included for summarizing IDH1-associated phenotypes. Main Outcome Measures Tumor DNA was sequenced by next generation sequencing analyzing a customized panel of genes. Results The overall prevalence of IDH1 hotspot variants in the main cohort was 0.5% (6/1141). Among those PPGLs without mutations in 15 common driver genes, the prevalence of IDH1 variants was 0.9% (4/455). When restricted to PGLs without mutations, the prevalence reached 4.7% (4/86). Among UBPGLs, IDH1 hotspot variants accounted for 8% (4/50). Together, all ten patients (9 PGLs and 1 PCC) with IDH1 hotspot variants, including three females with concurrent EPAS1 hotspot variants, had apparently sporadic tumors, without metastasis or recurrence. There were three patients with biochemical data, all showing a non-adrenergic phenotype. Conclusions The somatic IDH1 hotspot variants causes PPGL development in some Chinese patients, especially among those apparently sporadic PGLs with a non-adrenergic phenotype and without mutations in major PPGL driver genes.