1. INTRODUCTIONAntiphospholipid Syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, namely, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or antibodies to the protein cofactor-β2 glycoprotein I (anti-β2GPI) which cause a hypercoagulable state resulting in recurrent venous, arterial or small vessel thromboembolism and/or pregnancy-related complications. Thrombus can be either venous (59%) or arterial (28%) or both arterial and venous (13%). It was first described by Hughes in 1983, hence also known as Hughes Syndrome. In 1992, another subset of APS (<1%), termed Catastrophic APS, was identified where multiple small vessels supplying major organs infarct within a short period of time leading to multi-organ failure and high mortality (>50%). APS is far more prevalent in women than men in the adult age group (5:1) whereas in the pediatric population, the ratio is 1:1. This paper reports a rare case of primary antiphospholipid syndrome in a young male who presented with acute digital ischemia and was successfully treated with a combination of anticoagulation and glucocorticoid.