We present a rare case of fibromuscular dysplasia (FMD) manifesting in the mid to distal segment of the left anterior descending (LAD) artery, which led to the development of acute coronary syndrome (ACS) in our patient, highlighting the severe consequences of this vascular disorder. During the investigation of the patient's clinical symptoms, an unexpected incidental finding emerged, indicating bilateral FMD involvement of the renal arteries. This serendipitous discovery underscores the importance of comprehensive evaluation and thorough exploration when managing patients with FMD. We aim to shed light on the intriguing nature of FMD and emphasize the need for vigilant assessment to identify potential multi-vessel abnormalities, even beyond the primary affected site. We also aim to highlight the coronary artery manifestation of FMD as ACS and discuss its medical management.