Objective: Holmes tremor (HT) is a rare movement disorder. HT may also be associated with other neurological symptoms, including cognitive dysfunction. This study aims to investigate the pathophysiological mechanisms, diagnostic approaches, and therapeutic strategies for HT, thereby providing guidance for clinical management. Methods: The diagnosis of HT primarily relies on clinical features and neuroimaging techniques, such as MRI. Treatment options include pharmacological interventions (e.g., levodopa, trihexyphenidyl) and surgical approaches (e.g., deep brain stimulation, thalamotomy). A systematic review of the literature was conducted to summarize the clinical features, pathophysiological mechanisms, and treatment outcomes of HT. Results: HT symptoms typically emerge between 4 weeks and 2 years after neurological injury, associated with abnormal synaptic reorganization and collateral axonal sprouting following neuronal damage. The efficacy of pharmacological treatments varies among individuals, with levodopa showing effectiveness in approximately 54% of patients, though overall efficacy remains limited. Other medications, such as antiepileptic drugs and dopamine agonists, are also commonly used, but their effectiveness can be highly variable, with some patients showing poor response to these drugs. Surgical interventions, including deep brain stimulation, thalamotomy, lesionectomy of the original pathological focus, and ablation of specific nuclei, have demonstrated some efficacy but exhibit individual variability. There is currently no unified treatment consensus, and large-scale multicenter studies validating the long-term outcomes of novel therapies are lacking. Conclusion: The treatment of HT remains challenging, and its pathophysiological mechanisms are not yet fully understood. Future research should further investigate the pathophysiological mechanisms of HT, particularly its relationship with cognitive dysfunction, and optimize treatment strategies. Conducting multicenter studies to validate the long-term efficacy of novel therapies holds promise for improving the quality of life and prognosis of HT patients.