Early infantile epileptic encephalopathy is a severe form of epileptic encephalopathies which is characterized by the onset of generalized or lateralized tonic spasms within 3 months of life. These spasms are not dependent on sleep cycle and can occur hundreds of times per day, which may lead to psychomotor impairment and death. EIEE may be caused by different etiologies. Structural brain abnormalities, metabolic disorders or genetic abnormalities in certain genes can lead to EIEE. Interictal ‘Suppresion Burst’ EEG pattern is characteristic of EIEE. We are reporting this infant who showed neurodevelopmental delay and severe psychomotor impairment. He received inj. ACTH therapy and oral steroids along with multiple anticonvulsants, which showed only marginal improvement.  He was diagnosed as having Early Epileptic Encephalopathy-3, due to SLC25A22 gene, (also named ‘GC1’, MIM #609302, NM_024698), which encodes a mitochondrial glutamate carrier, responsible for an autosomal recessive form of neonatal epileptic encephalopathy, hence parents were studied for the gene defect, although they were asymptomatic. In both the parents, SLC25A22 gene was detected on exon 2 on chromosome11 in heterozygous form. Parents were counselled regarding poor prognosis of baby. At the age of 15 months of life, baby succumbed to death.