Objective To study the profile of neurological manifestation of rickettsial disease in children. Methods Review of hospital records was done in a tertiary care hospital for the period from January to December, 2020. Data of all the children fulfilling the inclusion criteria i.e., clinical criteria and serology were retrieved from the hospital records. Results Of the total 7974 children admitted over this period, 178 were diagnosed with rickettsial disease wherein 54 (33.3%) had neurological involvement. Convulsions (59%), altered sensorium (56%), headache (44%), meningeal signs (37%), ataxia, (11%), lateral rectus palsy (7.5%) and stroke (7.5%) were the major neurological manifestations. Cerebrospinal fluid (CSF) analysis done in 30 (55%) children showed pleocytosis [median (IQR) cells 15 (3.75, 50)] with lymphocyte predominance [median (IQR) lymphocytes 11.5 (3, 38.75)] and elevated proteins [median IQR 41.5 (29.75,61)]. Neuroimaging abnormalities noticed were cerebral edema ( n =7), cerebellar hyperintensities ( n =5), basal ganglia infarcts ( n =2) and hippocampal hyperintensities ( n =1). Conclusion Early recognition of rickettsial infection as a cause of neurological manifestation would facilitate early specific management.