Hirayama Disease (HD) is a benign and self-limiting motor neuron disease that mainly affects adolescent males, but its pathological mechanism is far from "benign". The core lies in the dynamic spinal cord compression and microcirculation disorder caused by cervical flexion. This article aims to conduct a systematic review of the global research progress of Hirayama disease, thoroughly exploring its epidemiological characteristics, complex pathophysiological mechanisms, clinical manifestations and classification evolution, the gold standard for diagnosis, and the optimal selection of treatment strategies. We have integrated 30 authoritative English literatures up to 2024, focusing on analyzing the quantitative diagnostic value of dynamic magnetic resonance imaging (MRI), the guiding significance of the Huashan classification system for individualized treatment, and the long-term efficacy comparison between anterior and posterior surgeries. In addition, this article also delves deeply into emerging genetic discoveries, potential serum biomarkers (such as neural filament light chains), and the challenges and opportunities faced in future directions such as stem cell therapy. This review indicates that through the integrated diagnostic model of "clinical - imaging - electrophysiology" in a trinity, early identification and intervention of Hirayama disease have become possible, thereby effectively preventing disease progression and improving the long-term prognosis of patients. Future research should focus on large-sample and multi-center collaboration to reveal its molecular mechanism and promote the development of precision medicine.