Introduction <p>Moyamoya disease (MMD) has a chronic nature which affects physical, cognitive, emotional challenges and relations with others reflecting on the quality of life (QoL) of affected children. Counseling is considered an important supportive intervention that may help children with their caregivers cope with the disease.</p> Aim <p>This study aims to evaluate the effect of counseling on quality of life among children with MMD and knowledge of their caregivers. Caregiver outcomes were limited to knowledge assessment.</p> Methods <p>A quasi-experimental single-group using paired t-test for pre–post study was conducted at a neurovascular outpatient clinic affiliated with a pediatric hospital in Egypt. A convenience sample of 30 children diagnosed with Moyamoya disease and their caregivers were included. Data were collected prior to counseling and after completion of counseling using a structured questionnaire assessing demographic characteristics and caregiver knowledge, as well as the Pediatric Quality of Life Inventory (PedsQL) for children. PedsQL was transformed to 0–100 scale where higher score means better quality of life.</p> Results <p>Before counseling, children demonstrated lower scores PedsQL across health and activities, child feelings, relations with others and school functions. Following the counseling, a statistically significant improvement in PedsQL scores was observed. In addition, caregiver knowledge significantly improved after counseling. A positive correlation was found between children’s PedsQL scores and caregiver knowledge.</p> Conclusion <p>Counseling may be associated with improvements in the QoL of children with MMD and enhancement of caregiver knowledge. Further studies using controlled designs and larger samples are recommended to confirm these findings.</p>