This article aims to summarize the experience in the nursing care of an 8-year-old child with sitosterolemia. The mainly nursing points are as follows:to formulate a personalized diet list,to restrict the intake of plant sterols and some animal sterols;to monitor the growth and development indicators and assess nutritional status;to observe and record changes in xanthomas and reduce their disturbance;to closely observe the condition to prevent complications;to strengthen psychological care for the child and family members to relieve their negative emotions;to conduct regular follow-up and continued care to improve disease management capabilities of the child and his family. After 20 months of continuous follow-up,the patient’s condition improved without any complications.