GAD-positive rhombencephalitis is a rare autoimmune neurological disease caused by antibodies against glutamic acid decarboxylase (GAD), which regulates neurotransmitters. The case report describes a 43-year-old patient with progressive symptoms such as unsteady gait, cranial nerve palsy, dysphagia, dysarthria and hydrophobia. Diagnostics confirmed slightly increased serum anti-GAD antibodies, brain MRI showed mild cerebellar atrophy and EEG multifocal slowing with sharp waves in the bitemporal and central region without epileptiform discharges. Investigations ruled out infectious, metabolic, paraneoplastic and other autoimmune causes. Initial treatment with pulse corticosteroids and IVIG provided partial relief, but the patient developed respiratory distress and pneumonia requiring intensive care. Additional immunotherapy included high-dose corticosteroids and plasmapheresis with management of complications. The case highlights the rare manifestation of hydrophobia and the need for a multidisciplinary approach.