The prevalence of thrombophilia and dyslipidemia among young survivors of acute coronary syndrome has not been clearly defined. The purpose of the current study was to investigate the prevalence of multiple markers of thrombophilia and dyslipidemia in a cohort of consecutive young survivors of acute coronary syndrome. The study cohort included 156 consecutive young patients (men <45 and women <50 years), admitted to the intensive cardiac care unit with newly diagnosed acute coronary syndrome. Analysis included baseline, clinical and epidemiological characteristics, angiographic coronary anatomy, echocardiographic evaluation, extensive lipid and thrombophilia laboratory profiles, and in-hospital and 1-year clinical outcomes for all patients. Acute myocardial infarction was diagnosed in 142 (92 %) patients, of whom 108 (72 %) had ST-segment elevation. Eighteen (12 %) patients had no traditional risk factors. Low levels of high-density lipoprotein (<40 mg/dL) were found in 101 (65 %) patients, and 49 (34 %) patients had elevated levels of lipoprotein(a) (Lp(a)) (>30 mg/dL). Eighteen (12 %) patients were diagnosed with antiphospholipid antibody syndrome (APS), and 73 (47 %) had at least one laboratory finding consistent with thrombophilia. Patients with APS had significantly higher levels of Lp(a) (46 ± 32 vs. 29 ± 31 mg/dL, p = 0.005). APS is a common prothrombotic state found in young survivors of acute coronary syndrome. Lp(a) levels are elevated among APS patients who present with premature acute coronary syndrome.