Neurofibromatosis type 2 in pregnancy is an extremely rare condition. Central nervous system involvement with shwannomas and spinal tumours is very common. This condition is very challenging for obstetricians especially when the neurological complications starts manifesting. Close obstetric care, timely interventions and Multidisciplinary approach is the key to successful outcome in cases of Neurofibromatosis 2 in pregnancy.

Keywords: Neurofibromatosis Type 2; Pregnancy; Schwannomas; Paraparesis.