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Advances in multimodality imaging have enabled earlier detection and precise anatomical delineation, shifting the paradigm from incidental diagnosis to proactive management. This review synthesizes contemporary understanding of AAOCA pathophysiology, diagnostic strategies, and surgical management. The mechanisms of ischemia\u2014slit-like ostium, intramural course, acute take-off angles, and interarterial compression\u2014underscore the condition's dynamic nature. Diagnostic evaluation integrates anatomical imaging with computed tomography or cardiac magnetic resonance and functional assessment through perfusion testing to identify ischemia. Surgical intervention is indicated for high-risk anatomy or documented perfusion defects. Unroofing and coronary reimplantation remain the mainstays of therapy, while ostioplasty, pulmonary artery translocation, and coronary artery bypass grafting are reserved for anatomically or technically specific scenarios. Successful AAOCA management requires individualized surgical planning grounded in anatomical precision and physiologic understanding. Long-term surveillance with multimodality imaging and stress testing is essential, as residual or delayed complications\u2014such as restenosis, fibrosis, or aortic insufficiency\u2014may occur. With accurate diagnosis, meticulous surgical execution, and disciplined follow-up, most patients achieve excellent outcomes. 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