Chronic meningitis is an inflammation of the meninges with subacute onset and persisting cerebrospinal fluid (CSF) abnormalities lasting for at least one month. Several non-infectious and infectious etiologies are known to be causative. The wide range of different etiologies renders the approach to patients with this syndrome particularly difficult. There is no standardized diagnostic procedure, thus, taking an in depth history combined with a complete physical examination is mandatory in every patient.This review aims to present the current knowledge on etiology, neurological course of disease, diagnostic and therapeutic management steps of patients presenting with clinical signs and symptoms of chronic meningitis and meningoencephalitis. Still, the etiology of one third of patients remains unclear, reflecting the diagnostic challenge of this syndrome for each physician or neurologist, respectively. However, most patients with idiopathic chronic meningitis have a relatively good outcome.