Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurodegenerative disorder caused by persistent aberrant measles virus infection. It is characterized by behavioral changes, cognitive decline with deterioration in academic performance, visual dysfunction, focal or generalized seizures ,myoclonus, spasticity, mutism and akinesia ultimately leading to a vegetative state. Balint's syndrome, characterized by the triad of simultagnosia, optic ataxia, and oculomotor apraxia, as an initial presenting feature of SSPE is rare. Autonomic dysfunction in SSPE is attributed to central autonomic involvement, with decreased heart rate variability a predictor for arrhythmia and sudden cardiac death. We report an unusual case of a 22-year-old male presenting with features suggestive of Balint's syndrome. Myoclonus and cognitive decline appeared 6 months after the first onset of symptoms, along with autonomic dysfunction. Thereafter, rapid progression of symptoms was noted. Cerebrospinal fluid and electroencephalography after the first symptom onset were consistent with a diagnosis of SSPE. The patient ultimately succumbed to his illness. Thus we highlight atypical presentation of SSPE with autonomic dysfunction. A high index of suspicion is needed for prompt and timely intervention.