Histopathology and immunohistochemistry confirmed the presence of a skull-base paraganglioma.<h4>Conclusions</h4>Succinate dehydrogenase complex subunit D mutation-associated bilateral carotid body tumors with a concomitant skull-base paraganglioma accompanied by abnormal dopamine and hypertension are extremely rare, which not only provides ideas for considering the association of gene mutations, biochemical abnormalities and clinical symptoms but also provides an expanded diagnostic spectrum for paraganglioma in atypical locations. The gene discussed is SDHD; the disease is Hypertension.