A 35-years-old right-handed Hindu male with no known comorbidities presented to neurology out-patient department (OPD) with weakness of ipsilateral (left) upper and lower limbs for three months. The weakness gradually progressed to left sided hemiparesis. He denied of facial deviation, loss of consciousness, drooling of saliva, abnormal body movements, any history of trauma lethargy and fatigue. There was no any relevant medical and surgical history. Family history was found to be insignificant. On general examination, he was well-conscious and oriented to time, place and person. The vital parameters were stable. His higher mental functions were intact. The motor examination revealed power of 1/5–3/5 in medical research council (MRC) grading on left upper and lower limbs. The tone of left lower limbs was decreased as compared to the upper limbs. The lower limb had findings such as atrophy and fasciculations. The deep tendon reflexes were brisk on left upper limbs and lower limbs. There was foot drop in left side with positive Hoffman sign. The plantar reflex was upgoing on left side. There was no any sensory involvement. The bowel and bladder examination revealed no any abnormalities. The rest of the systemic examination was sound and intact. He was admitted for further evaluation. The baseline investigations including complete blood count, renal and liver function tests, random blood sugar and electrolytes were within reference range. The magnetic resonance imaging (MRI) of the brain did not reveal any abnormalities as shown in. It revealed normal brain morphology with normal parenchymal signal intensity. The areas of internal capsule and brain stem had normal signal intensity. There was no any evidence of infarct or hemorrhages. Similarly, screening MRI of whole spine and brachial plexus was performed which did not have any significant findings except for mild degenerative changes in cervical and lumbar spine. Later during the hospital stay, he developed tingling and burning sensation in his left medial forearm area. The nerve conduction study showed abnormal motor nerve conduction velocities (MNCV) in left median, ulnar and common peroneal nerve suggesting moderate axonal loss with demyelination. This was suggestive of motor neuron disease (MND). The differentials considered were peripheral neuropathy, vitamin-B12 deficiency, thyroid disease, ischemic stroke, hereditary spastic paraplegia, myasthenia gravis and amyotrophic lateral sclerosis (ALS). The other possibilities were ruled out based on the history and clinical examination. He was then diagnosed ALS on the basis of Gold Coast criteria [,, ]. On exome sequencing [], an atypical gene was isolated from gene sequencing as shown in. The variant coverage statistics for TFG gene included reference allele coverage-G = 67 and alternate allele coverage-C = 57. The percentage target nucleotides covered included depth ≥20X, 93.97% coverage and quality threshold of 98.01%. The treatment was started with edaravone with the initial cycle of 60 mg once daily for 14 days, followed by a 14-day drug free period. The subsequent cycles constituted 60 mg once daily for 10 days within a 14-day period, followed by a 14-day drug free period. Pregabalin and sertraline were also given as a supplementary treatment. He accepted the treatment and was discharged with the advice of follow-up for next edaravone cycle. He was followed-up during the edaravone cycle clinically. After completing four cycles of the edaravone treatment, he left the treatment. The clinical status was unchanged with some of the new symptoms like swallowing difficulty.