A 13-month-old male presented with repetitive episodes of myoclonic jerks of the head and extremities for two months. His past medical history revealed that his non-immunized mother had measles at the time of delivery. Measles antibody titers in serum and cerebrospinal fluid (CSF) were 1/512 (hemagglutinin inhibition [HI]) and 1/128 HI, respectively. Immunofixation electrophoresis of CSF revealed an oligoclonal IgG band. The magnetic resonance imaging (MRI) of the brain on T2-weighted images showed lesions of high signal intensity in the subcortical white matter. Electroencephalography (EEG) revealed periodic high-amplitude slow waves. Diagnosis of subacute sclerosing panencephalitis (SSPE) was based upon clinical presentation, a characteristic EEG, and abnormal CSF studies. MRI findings supported the diagnosis. To the best of our knowledge, this is only the 3rd case to date, of SSPE-associated congenital measles in the literature.