Lyme disease is the most common vector-borne disease in the northern hemisphere. Neurological complications usually manifest in patients who do not receive treatment for Lyme disease. Neurological involvement may be early or late, depending on the duration of the symptoms. Early neuroborreliosis presents with symptoms such as headache and meningism; late neuroborreliosis can present with signs and symptoms of encephalopathy and stroke-like symptoms. The diagnosis is based on clinical manifestations and lumbar puncture finding. Treatment consists of intravenous antibiotics for a period of three to four weeks. Patients who receive early treatment usually have an excellent prognosis, with very few patients developing post-treatment Lyme disease syndrome. Here, we report an unusual case of Lyme disease with extremely high cerebrospinal fluid protein level and devastating neurological sequelae. The diagnosis of neuroborreliosis is based on neurological symptoms and lumbar puncture findings.