BACKGROUND: Vaginal paraganglioma are rare, atypical, solitary tumors which originate from the female genital tract. Sacrococcygeal teratoma are also rare neoplasms which derive from one (or more) primordial germ cell layers. Here we report a unique case of vaginal paraganglioma with sacrococcygeal teratoma. CASE PRESENTATION: A 44-year-old female experienced paroxysmal hypertension, palpitations and dizziness for almost six years. Enhanced CT and MRI highlighted two abnormal soft tissue lesions located in the left vaginal wall and coccyx anteriorly, and Iodine-131 metaiodobenzylguanidine (131I-MIBG) demonstrated abnormal radioactive uptake in perineum area. Endocrine tests showed elevated plasma normetanephrine (NMN) and 24 h urine norepinephrine. There was a well-circumscribed soft tissue lesion of approximately 3.5 cm in the left lateral vaginal wall which could be palpated during bimanual examination, together with a 1.5 cm tumor in the posterior wall of the rectum. We completely resected the two lesions in stages with the support of a senior gynecologist and general surgeons. Postoperative histopathological examinations suggested the vaginal paraganglioma and mature sacrococcygeal teratoma. Targeted sanger sequencing for the 36 mostly common paraganglioma-related genes, with a depth of 1000x, revealed no mutations. Post-operatively, plasma NMN and 24 h urine norepinephrine returned to the normal range and her symptoms completely disappeared. CONCLUSIONS: We reported an extremely rare case and the successful treatment of functional vaginal paraganglioma coexisting with adult sacrococcygeal teratoma.