Rare neurological diseases account for approximately 60% of all rare diseases, representing a category with a relatively large patient population and wide-ranging impact. These disorders are highly heterogeneous, with complex clinical manifestations that may involve neuromuscular, metabolic, immune, or developmental pathways. As a result, they are difficult to diagnose and prone to misdiagnosis. Currently, there is no internationally unified classification standard specifically for rare neurological diseases. In clinical practice, these conditions often face challenges such as delayed diagnosis and limited access to treatment. This article reviews the classification, diagnosis, and treatment of rare neurological diseases, aiming to provide clinical reference.