<p>Rabies encephalitis is a fatal zoonotic disease that poses diagnostic challenges due to its variable clinical presentation. This report describes a pediatric case involving an 8-year-old boy who presented with rapidly progressive neurological decline following unrecognized exposure to a rabid cow in a quarantined village, a rare but possible route of transmission. Although initial cerebrospinal fluid and saliva polymerase chain reaction assays for Lyssavirus were inconclusive, early magnetic resonance imaging (MRI) revealed characteristic involvement of the basal ganglia and limbic system. These characteristic neuroimaging patterns prompted further investigation with a nuchal skin biopsy, which confirmed the diagnosis by direct fluorescent antibody staining. Despite aggressive supportive care, the patient’s condition progressed to classic hydrophobia and autonomic instability, ultimately leading to death 8 days after admission. This case highlights the role of early, sequential MRI as a valuable adjuctive diagnostic tool in rabies encephalitis, particularly when molecular tests fail to provide immediate confirmation. It underscores the importance of maintaining a high index of suspicion based on exposure history and suggestive imaging findings to facilitate early recognition of this lethal neurotropic infection.</p> Graphical Abstract <p></p>