PURPOSE OF REVIEW: This article reviews the symptomatology, diagnosis, and treatment of neuroborreliosis.RECENT FINDINGS: The most recent guidelines for the diagnosis and treatment of Lyme disease were published in 2020 by the Infectious Diseases Society of America, the American Academy of Neurology, and the American College of Rheumatology. SUMMARY: The most common neurologic complications of Lyme disease are cranial neuritis (most often a unilateral or bilateral facial nerve palsy), meningitis, and radiculoneuritis/mononeuropathy multiplex. Testing for Lyme disease begins with an enzyme-linked immunosorbent assay (ELISA). If the ELISA is positive or borderline, Western blots should be performed for both IgM and IgG antibodies. As a general rule, in infectious diseases, an IgM antibody response is followed by an IgG antibody response. A central nervous system infection has either a CSF pleocytosis or pathogen-specific intrathecal antibody production. Lyme meningitis, cranial neuropathy, radiculoneuropathy, or other peripheral nervous system manifestations are treated with oral doxycycline or IV ceftriaxone, cefotaxime, or penicillin G. No additional antibiotic therapy is indicated for patients with posttreatment Lyme disease syndrome or patients with concern for chronic Lyme disease with no evidence of previous or current Lyme infection.