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Leigh’s syndrome or sub-acute necrotizing encephalopathy is a rare progressive neurodegenerative disorder of paediatric age group. It has variable clinical, imaging and pathological presentation. Typical MRI features lead to the diagnosis. It is usually of infantile or juvenile form, which presents in <5 years of age. Here, the present authors report an unusual case of 7-year-old male presented with complaints of frequent falls, slurring of speech, difficulty in walking and regression of the achieved milestone was noted. MRI brain study showed altered signal intensity in bilateral putamen, bilateral caudate nucleus, midbrain, substantia nigra, periaqueductal region and pons. Magnetic Resonance spectroscopy revealed high lactate levels in pontine lesions. MRI findings were suggestive of subacute necrotizing encephalopathy.