The Dyke-Davidoff-Masson syndrome is a rare central nervous system disease. In clinical practice, it is necessary to combine complete medical history data and timely use imaging techniques such as cranial CT or MRI for clinical diagnosis of this disease, so as to help patients select better treatment options in a timely manner and reduce the number of misdiagnoses and relapses. In the present study, we undertake a comprehensive analysis of the diagnosis and treatment of one clinical case of Davidoff-Masson syndrome, and referencing pertinent literature to enhance the depth of clinical analysis and furnish a valuable reference for clinical practice.