Acute necrotizing encephalopathy (ANEC) is a rapidly progressive neurologic disorder that occurs in children after common viral infections such as influenza A and herpes simplex virus. It is observed more commonly in Asian countries. Magnetic resonance imaging findings in ANEC include symmetrical brain lesions, preferentially affecting the thalamus bilaterally. However, similar neuroimaging findings are also observed in deep cerebral vein thrombosis, which can lead to misdiagnosis. We report a case of 2½-year-old child who presented like acute necrotizing encephalopathy but on further investigations was found to be having deep cerebral vein thrombosis.