Aim: Several neurological disorders occur in women of the childbearing age group and are an important cause of maternal morbidity and mortality. The diagnosis and management of neurological disorders in pregnancy is always a challenging task due to varied symptomatology and risks to the fetus. A rare neurological disorder in pregnancy can be pregnancy related or can be caused by exacerbation of a pre-existing neurological condition or sometimes can be detected for the first time during pregnancy which might be an incidental finding. Treating these patients remain a challenge. Thus, our study aimed to determine the challenges in managing pregnant mothers with rare neurological complications in a tertiary health care hospital. Methods: All pregnant women with rare neurological conditions admitted to the Department of Obstetrics & Gynaecology, St. Johns Medical College and Hospital, Bengaluru, Karnataka, India during our 1-year study period (from January 2020 to December 2020) were analyzed retrospectively. Our experience may help to take proper decisions when a similar condition is encountered elsewhere. Results: In a study period of 1 year, the total incidence of neurological disorders with pregnancy was 0.89%, which included eclampsia (0.4%) and epilepsy (0.25%). We had nine cases with a rare neurological disorder. Three of them were primigravida (33.5%). Four were diagnosed with the neurological disorder before the pregnancy. Five cases were diagnosed for the first time during pregnancy. We had two cases with Wernicke's encephalopathy (WE), one case of the acute motor sensory axonal neuropathy (AMSAN) variant of Guillain–Barré syndrome (GBS), and one case with GBS. One case with right cerebellopontine angle tumor which got diagnosed during her first trimester of pregnancy. Four cases with pre-existing neurological disorder included status epilepticus, limb–girdle muscular dystrophy, middle cerebral artery (MCA) aneurysm/internal carotid artery (ICA) supraclinoid occlusion and situs inversus totalis with neuromyelitis optica. The mean maternal age was 27.77 years. Gestational age varied from case to case at the time of their presentation and five cases had associated obstetric complications. Six mothers recovered well, one mother went against medical advice, two mothers lost to follow-up and we had one maternal mortality. Conclusion: Even though knowledge about rare neurological diseases in pregnancy is finite, these cases require tertiary care referral and patients should be promptly managed through a multidisciplinary approach. Strict antepartum vigilance is essential for the treatment and good neonatal outcomes. Holistic and vigilant care toward these patients reduces mortality and improves maternal and neonatal outcomes.