This patient presented with progressive weakness and wasting of the lower limbs over 2-3 years along with tingling and numbness of the feet. On examination, he had ataxic gait, impaired sensation, and signs of cerebellar dysfunction. MRI showed cerebellar and spinal cord atrophy. Based on the early onset, family history, clinical features involving the cerebellum, posterior and lateral columns, diagnosis of Friedreich's ataxia was made.