Fragile X syndrome (FXS) is noted to be the leading cause of inherited intellectual disabilities and is caused by expansive cytosine-guanine-guanine (CGG) trinucleotide repeats in the fragile X mental retardation 1 gene (FMR1). FXS can display a wide range of behavioral problems in addition to intellectual and developmental issues. Management of these problems includes both pharmacological and nonpharmacological options and research on these different management styles has been extensive in recent years. This narrative review aimed to collate recent evidence on the various management options of behavioral problems in FXS, including the pharmacological and non-pharmacological treatments, and also to provide a review of the newer avenues in the FXS treatment.