Background: Autism spectrum disorder is a neurodevelopmental disorder associated with symptoms that include persistent deficits in social communication and social interaction across multiple contexts and restricted, repetitive patterns of behavior, interests, or activities. Measuring health- related quality of life is very important for children with autism spectrum disorder. Aim of the study: This study aimed to assess the quality of life in children with autism spectrum disorder. Design: A descriptive design was utilized in carrying out this study. Setting & Sampling: A purposive sample of 146 children suffering from autism and their mothers were attending at Institute of Childhood Studies that affiliated to Aim Shams University and the Out-patient Child Neurology Clinic that affiliated to Sohag University Hospital. Tool: Interviewing questionnaire sheet composed of 3 tools; tool I: Child's assessment sheet, tool  II: Mothers' reported practices, tool  III: Quality of life questionnaire. Results: It was found that, there is high statistically significant positive correlation between total studied mothers' practices and their children quality of life. Also, there was statistically significant positive correlation between total studied mothers' practices and their total knowledge. In addition' there is statistically significant positive correlation between total studied mothers, knowledge and their children quality of life. Conclusion: Based on the findings of the present study, it was concluded that, more than two fifths of the studied mothers had average level of reported practices regarding their role towards their children. In addition, there was high statistically significant positive correlation between total studied mothers practices and their children quality of life. Recommendation: Encourage continuous sharing of the mothers during the care of their children with autism by specialists to identify needs and problems of ASD.