Antiphospholipid antibody syndrome (APS) has a broad spectrum of thrombotic and non-thrombotic clinical manifestations. We present a case of APS presenting with seizure, stroke and atrial mass. A 38 year-old male presented with headache of 10 days duration and tonic-clonic seizure. Neurological examination was normal. MRI brain showed small acute right cerebellar infarct. MRA brain and neck showed focal narrowing in origin of internal carotid artery bilaterally. EEG was normal. He was started on aspirin, atorvastatin and carbamazepine. Transthoracic and transesophageal Echo showed atrial mass pedunculated and lobular measuring 1 * 1.5 cm freely mobile across mitral valve opening across the left ventricular inflow. Antiphospholipid (aPL) antibodies were positive in higher titer (IgG> 120). B2 glycoprotein was 90. ANA was negative. ESR and CRP were normal. Platelet count was low. APS was diagnosed. He underwent successful surgical removal of the mass, which looked like a thrombotic clot, Histopathological analysis confirmed fibrinous clot, with no evidence of tumor cells. He was started on full anticoagulation treatment and was followed up regularly in the clinic, where he did not have any further complications from the disease. Our patient was diagnosed to have APS based on the features of high positive anticardiolipin antibody IgG and B2 glycoprotein IgG, Stroke, thrombocytopenia and abnormal ECHO findings. Thrombotic vegetation can mimic an atrial myxoma on ECHO. APS can present with neurological and cardiac manifestations and therefore high index of suspicion is necessary for diagnosis.