Superior oblique myokymia (SOM) is a rare disorder characterized by episodic monocular torsional oscillations, often caused by vascular compression of the trochlear nerve. This condition can significantly impair daily life, yet definitive treatment options remain limited. In this study, we reviewed the clinical outcomes of seven patients, including two treated at our institution, who underwent microvascular decompression (MVD) for SOM. Preoperative 3D fusion imaging was instrumental in identifying neurovascular compression, leading to immediate and complete symptom resolution in all cases, with no recurrences during the follow-up period. Our findings highlight the effectiveness of MVD for refractory SOM and underscore the importance of collaboration between ophthalmologists and neurosurgeons to optimize patient outcomes.