Rationale: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.Patient concerns: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.Diagnoses:Based on the biopsy results, including immunohistochemical and electron microscopic findings, and molecular studies, her condition was diagnosed as intraneural perineurioma.Interventions: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.Outcomes: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.Lessons: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.