Background: One of the most popular motor neurons diseases, amyotrophic lateral sclerosis (ALS) affects almost 140 thousand people per year globally. Studies show that this neurodegenerative pathology is commonly associated with mutations in the dismutase superoxide-1 (SOD-1) protein – reason why recent treatments objectify reducing its synthesis. The primary drugs used for this purpose are the antisense oligonucleotides, which have Tofersen as its recently FDA approved representative. While still controversial, the drug has shown positive effect against motor symptoms and slowed disease’s progression. However, several studies have also noticed dangerous side effects associated with its use. Objective: This review aims to comprehend Tofersen’s adverse effects during treatment of ALS and to ponder risk-benefit ratio. Results: The main adversities related to Tofersen’s use were myelitis, lumbar radiculopathy, aseptic meningitis, intracranial hypertension and papilledema – observed in patients submitted to clinical experimental trials; although some were spontaneously resolved, others only disappeared after discontinuing the drug’s infusion. A confusion factor appointed was that the disease itself could contribute to neural system inflammation and may not have 100% correlation to the medication, but cerebrospinal fluid analysis after Tofersen’s administration showed pleocytosis and higher protein levels and, in addition, patients who received placebo didn’t present these effects – which contributes to associating Tofersen as their cause. Conclusions: Even though research has shown that there is a significant probability of Tofersen causing severe neurological adverse effects, most of the patients who experienced these symptoms had spontaneous recover with no further complications; which leads to believing that the impact of this drug in the progression of ALS so far has more benefit of use than its risk for negative consequences.