A heterozygous missense variant in SLC25A11 (c.715C>A/p.Pro239Thr) was first identified in a 46-year-old patient with a non-secreting abdominal paraganglioma, which exhibited a cluster 1A molecular profile in the absence of pathogenic variants in SDHx or FH [18]. Here, SLC25A11 is linked to paraganglioma.