Background <p>Autosomal dominant polycystic kidney disease is the most common hereditary kidney disorder, characterized by the progressive formation of renal cysts due to genetic mutations. In addition to kidney involvement, autosomal dominant polycystic kidney disease is associated with various extrarenal complications, including cysts in other organs, cerebral aneurysms, and cardiac lesions. Although less common, coronary aneurysms and dissections can also occur, an association that will be explored in this study.</p> Case presentation <p>This report describes a 50-year-old, Caucasian male patient with autosomal dominant polycystic kidney disease who was previously submitted for a kidney transplant and later developed coronary aneurysms and dissections. Subsequently, the patient was submitted for myocardial revascularization and multiple attempts to restore cardiac vasculature.</p> Conclusion <p>The patient developed hemodynamic instability and died 2&#xa0;days after the final procedure. Managing such a patient is complex, and prevention and continuous monitoring are essential to guide the best surgical approach.</p>