Tuberculosis (TB) is a disease of global concern due to its varying clinical presentations and outcomes. Hemophagocytic lymphohistiocytosis (HLH) syndrome, along with obstructive jaundice, is one of the rarest presentations of tuberculosis involving immune activation and has a very high mortality rate. Thus, on-time diagnosis becomes crucial for the management of the disease. Prompt treatment with anti-tubercular therapy (ATT) can limit the morbidity and mortality associated with it.We report the case of a 28-year-old male who presented with fever, yellowish discoloration of the skin, features of bicytopenia, jaundice with hepatosplenomegaly, and ascites. The liver function test (LFT) was suggestive of obstructive jaundice. TB was confirmed on the analysis of lymph node aspirates, and the contrast-enhanced computed tomography (CECT) of the thorax and abdomen was suggestive of disseminated tuberculosis. Upon investigation, the criteria for HLH were fulfilled. Bone marrow aspiration smears revealed multiple hemophagocytic histiocytes in the background of a hypercellular marrow, erythroid hyperplasia, and myeloid-to-erythroid ratio of 1:1. Thus, a diagnosis of disseminated TB with HLH and obstructive jaundice was established. A modified ATT regimen was started, keeping in mind the deranged LFT of the patient, but no immunosuppressive therapy was initiated as it could make the TB worse. This case demonstrates the fact that in cases of hemophagocytic syndrome with tuberculosis as an underlying cause, just starting ATT without immunosuppression could be rewarding and lifesaving.