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Eye-tracking technology is an emerging tool to monitor eye gaze non-invasively. This technique has been adopted widely to investigate eye gaze during visual stimuli and tasks in health and disease. We reviewed studies that aimed to determine the application of an eye-tracker for individuals with Rett syndrome. A systematic search identified eight studies that were summarized in terms of main purpose, subject, study design, eye-tracking technology, main parameter, and primary finding of interest. The main purposes of the reviewed studies were to evaluate cognitive function, judge habilitation intervention outcomes, and demonstrate that both epilepsy features and electroencephalography characteristics are correlated with the ability to recognize and match pairs and categorize semantically with respect to animals and behavioural features. The eight studies included 193 individuals with Rett syndrome, ranging from 1.5 to 31 years of age. Four of the eight studies included typically developing female participants as a control group to compare eye gaze features. The reviewed studies could enable family members and care staff to know intentionality, recognize facial expressions and concepts of colour, shape, size, and spatial position, the strict correlation between neurophysiological features and neuropsychological impairment, preference for socially weighted stimuli and for novel and salient stimuli, attention and recognition memory, and habilitation outcomes. We suggest that the findings obtained by this technique should be utilized to evaluate and examine habilitation plans and means, judge intervention outcomes, and identify correlations between neurophysiological features and neuropsychological impairment and are of significant benefit to know the hidden and potential abilities of individuals with Rett syndrome.