Drug rash eosinophilia with systemic symptoms (DRESS syndrome) presents as an acute febrile illness with leukocytosis, eosinophilia, lymphadenopathy, skin rash with acute hepatitis, renal failure, myositis, or systemic organ involvement. Aromatic anticonvulsants like phenytoin, carbamazepine, and phenobarbital cause drug-induced hypersensitivity or DRESS syndrome. However, sodium valproate being nonaromatic compound although known hepatotoxic drug in preexisting chronic liver disease has never been reported to cause DRESS syndrome alone. Here we report an interesting case of DRESS syndrome caused by valproate, which presented as an acute hepatitis illness with rash, renal dysfunction, and typical hematological features of DRESS syndrome within 2 months of the introduction of the drug in an epileptic patient. Patient initially showed a good response to intravenous steroids with improvement in the liver and renal dysfunction. However, later on, developed pancytopenia either due to steroid-induced sepsis or DRESS syndrome-related secondary hemophagocytosis (HPS) due to involvement of bone marrow as a rare occurrence and succumbed to illness.