Background: Macrophage activation syndrome (MAS) associated with Primary Sjogren's syndrome is a rare entity. The following are our observations on a patient of Indian origin who presented with features of cholestatic jaundice and was diagnosed with MAS. We also discuss the challenges faced during its management. Case description: A 53-year-old woman presented with generalized weakness and yellowish discoloration of skin, sclera, and urine for 1 month. She was evaluated for various etiologies of acute hepatitis. During this, the autoimmune profile was suggestive of Primary Sjogren's syndrome. Despite starting appropriate treatment, her liver dysfunction worsened, and her fever persisted. Further workup revealed high ferritin, triglycerides, and lactate dehydrogenase (LDH). Secondary hemophagocytic lymphohistiocytosis (sHLH) was suspected, and biopsies from the liver and bone marrow were performed. Macrophages, lymphocytes, and histiocytes were present in the marrow. Hence, she was diagnosed with MAS associated with portosystemic shunts (PSS) with liver dysfunction. She was treated with steroids, etoposide, cyclosporine, and intravenous immunoglobulin (IVIg). Despite best efforts, there was no clinical improvement, and she was discharged against medical advice. Six weeks later, she succumbed to her illness at home. Conclusion: Macrophage activation syndrome is a rare entity. The mortality is very high, especially when initial ferritin levels are elevated and also if they do not respond to steroids.