Vigabatrin (VGB), a selective irreversible inhibitor of gamma-aminobutyric acid transaminase, has proved to be effective against cryptogenic and symptomatic infantile spasms (IS). Unfortunately, reports of serious visual field defects have led to a drastic reduction in the use of the drug. This review is based on a systematic search in the literature for evidence regarding efficacy and safety of VGB in IS. Based on a specific mechanism of action, there is a solid evidence of clinical efficacy of VGB in children with Tuberous Sclerosis. Similarly, VGB could represent a potential effective therapy also for spasms due to focal cortical dysplasia. In infants with spasms due to other causes, the risk of ophthalmologic toxicity should be carefully weighted against the benefit of controlling spasms.