Antiphospholipid syndrome (APS) is defined to be an acquired, systemic, and immunological disease. Budd-Chiari syndrome (BCS) appearing as a preliminary manifestation of APS is considered to be rare. Here the case of an 18-year-old boy is being reported. The patient had complaints of insidious onset painless abdominal distention for three months and further developed pedal edema. One month later he was found to have a low-grade fever without chills for which he was evaluated in a hospital where he was diagnosed with jaundice, ascites, and liver disease. On further evaluation, he was found to have proteinuria. He was then hospitalized at the gastroenterology specialty of Amrita Institute of Medical Sciences and Research Centre for further evaluation and management. Multi-detector computed tomography abdomen contrast was done and obstruction of all the three hepatic veins and a nodular non-homogenous enhancement of the liver confirmed the diagnosis of BCS. A hepatic venogram showed long segment occlusion with multiple collaterals around it. Hepatic vein angioplasty was done and was started on anticoagulation with warfarin and heparin. This case report gives an insight into the fact that health care professionals must take into account that even though it is not very common, they should not eliminate the probability that BCS can also occur as a primary manifestation of APS.