Background: Spontaneous coronary artery dissection (SCAD) has been reported to be a rare cause of acute coronary syndrome and sudden cardiac death. The clinical presentation of SCAD varies from asymptomatic to sudden death. Pregnancy is associated with SCAD, and autoimmune diseases, especially systemic lupus erythematosus (SLE), may play an important role in SCAD etiology. Case Report: A 37-year-old female with hypertension, SLE, a history of preeclampsia with 3 cesarean deliveries, and an active smoking habit presented to the emergency department with chest pain. On arrival, the patient was hypertensive with blood pressure of 152/122 mm Hg and a normal heart rate and respiratory rate. Given the patient's history of SLE and preeclampsia, antiphospholipid antibodies were tested. The anti-β2-glycoprotein 1 immunoglobulin G concentration was elevated at 30 U/mL, and lupus anticoagulant was positive. Electrocardiogram showed minimum ST elevation in lead V2. Initial troponin was 0.1 ng/mL, with a peak of 54.5 ng/mL after 6 hours. Aspirin 325 mg was administered, and the patient underwent urgent cardiac catheterization. Intravascular angiography showed evidence of intimal flap (mid left anterior descending artery) spontaneous dissection with subintimal hematoma. The angioplasty resulted in successful stent placement in the mid left anterior descending artery. Conclusion: SCAD diagnosis is challenging and requires a high index of suspicion. This case shows the challenge of early diagnosis of SCAD and highlights its association with autoimmune diseases, specifically SLE. Early recognition of this pathology results in better outcomes.