Objective To investigate the clinical characteristics of meningeal carcinomatosis with nervous system syndrome as initial symptom. Methods Clinical data, laboratory and imageing examinations were retrospectively analyzed in 23 patients of meningeal carcinomatosis from June 2009 to December 2017. Results Twenty patients (86.96%) had headache as initial symptom and 12 (52.17%) had headache with vomiting. Head enhanced MRI showed obvious enhancement in meningeal lesions, and 13 cases (59.09%) had meningeal enhancement with enlargement of ventricular system; CT showed enlargement of supratentorial ventricular system (1 case). About 65.22% (15/23) primary lesions were originated from lungcancer,and others were or iginated from colon cancer,hematological lymphoma, gastric cancer, spinal astrocytoma (4.35%, 1/23 respectively), and some unknown origin (17.39%, 4/23). About 82.61% (19/23) patients cerebrospinal fluid pressure increased obviously, Tumor markers especially carcinoembryonic antigen (CEA) were mostly increased in serum (11/17) and cerebrospinal fluid (7/10). Conclusions Headache and vomiting are the most common clinical symptoms of meningeal carcinomatosis. Enhanced MRI shows meningeal enhancement or communicating hydrocephalus. Cerebrospinal fluid pressure tends to increase significantly. Carcinoembryonic antigen is significantly increased in serum and cerebrospinal fluid. Primary lesions of meningeal carcinomatosis mostly originate fromlungcancer. Finding tumor cells in cerebrospinal fluid is the " golden standard" of diagnosis.