Neurosarcoidosis (NS) is a rare condition that may mimic central nervous system (CNS) infection, neoplasia and other inflammatory disorders of the CNS such as multiple sclerosis, encephalitis and vasculitis. Diagnosis is challenging in cases with minimal or absent systemic involvement. Cerebrospinal fluid (CSF) angiotensin-converting enzyme (c-ACE) has been claimed as a valuable diagnostic tool for NS. However, there is little data evaluating its performance in routine clinical practice.