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Intraneural perineurioma is an extremely rare form of peripheral nerve sheath tumor; and the anesthetic management of a parturient with intraneural perineurioma, especially affecting spinal roots and nerves of extremities, is very rare.A 28-year-old woman was referred to the hospital at 37+5 weeks’ gestation, presenting with a 10-year history of paroxysmal acroanesthesia and aching with distal limbs.Based on the biopsy results, including immunohistochemical and electron microscopic findings, and molecular studies, her condition was diagnosed as intraneural perineurioma.The size of pelvic nervous masses gradually increased with pregnancy. A scheduled elective cesarean section under general anesthesia was concluded for the patient under preoperative multidisciplinary consultation with anesthesiologist, neonatologist, and neurologist.The patient and the neonate were discharged smoothly on the fourth postoperative day. During a 6-month follow-up period, no new neurologic complication was observed.To our knowledge, this is the first case report that documented the anesthetic management for a parturient with intraneural perineuroma. Careful preconception care and multidisciplinary assessment are essential to achieve optimal reproductive outcomes.