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Background Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with thrombotic events, myocardial infarction, and valvular heart disease. During valvular replacement surgery, the high risk of thrombosis combined with the operative risks in these specific groups of patients poses a challenge to the medical team. Case presentation We present a case of a female patient with APS and mixed aortic valve disease. During surgery, she suddenly developed complete cardiac arrest. Three months later, after she recovered, and while she was still on close follow up, a thrombotic event caused myocardial infarction. After prompt and precise treatment, the patient successfully recovered; one year after surgery patient is doing well. Conclusion Adequate surgical technique along with optimal anticoagulation strategies and long term follow up are of paramount importance to ensure an uneventful recovery. A multidisciplinary team is required to manage these complex scenarios and high-risk patients.

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