Background: Balance and gait disorders have been observed in children and adolescents with sensorineural hearing loss (SNHL), justified by vestibular dysfunctions that these children may present, due to the injury to the inner ear. Therefore, some investigations have suggested that the practice of sports or recreational activities can improve the balance and gait of this population. Objective: Assess the evidence quality from randomized or quasi-randomized controlled trials that used sports or recreational activities as an intervention to improve the balance and /or gait of children and/or adolescents with SNHL. Methods: Systematic review that surveyed articles in nine databases, published up to January 10, 2019, in any language, using the following inclusion criteria: (1) Randomized or quasi-randomized controlled trials. (2) Participants from both groups with the clinical diagnosis of SNHL, aged 6–19 years old, without physical problems, cognitive or neurological deficits, except the vestibular dysfunction. (3) Using the practice of sports or recreational activities as an intervention, to improve the balance and/or gait outcomes. Results: 4732 articles were identified in the searches, after the removal of the duplicates articles and the reading of the titles and their abstracts, remained 16 articles for reading in full, being 5 trials eligible for this systematic review. Of the five eligible trials, three used sports activities and two recreational activities as intervention and presented very low-quality evidence for balance and gait outcomes. Significance: Sports and recreational practices seem to represent promising modalities to improve the balance and gait of children and adolescents with SNHL. However, due to the methodological limitations of the trials and the low quality of the current evidence on the topic, the results of the trials should be interpreted with caution. Due to the low quality of evidence observed, we suggest that new trials be proposed on this topic, with greater methodological rigor, to provide high-quality evidence on the effectiveness of sports and recreational practices to improve the balance and gait of children and adolescents with SNHL.