Abstract: Leptomeningeal metastasis (LM) is a fatal complication of advanced non-small cell lung cancer (NSCLC), with poor prognosis and rapid deterioration of performance status. In recent years, substantial progress has been made in key aspects such as the establishment of leptomeningeal-related clinical animal models, characterisation of genetic profiles, cytology and genotyping of cerebrospinal fluid biopsy, but the treatment of LM remains a difficult point in the diagnosis and treatment of NSCLC. Existing treatments include molecular targeted therapy, systemic chemotherapy, local radiotherapy, intrathecal chemotherapy, and immunotherapy, etc. However, the efficacy of LM is still poor. This review will focus on LM arising from NSCLC with a goal to provide the reader an understanding of the pathophysiology, pathogenesis, clinical presentation, diagnosis, treatment and prognosis.