The management of respiratory problems in children on home invasive mechanical ventilation (HIMV) is a complex and challenging task. In recent years, with appropriate family education, these patients have been able to be discharged from the hospital and continue their treatment at home. The population of pediatric patients dependent on HIMV has been increasing worldwide, presenting unique and varying care needs. Management of these patients involves addressing ventilator settings, monitoring respiratory status, ensuring airway safety, and providing continuous support and education to patients and their caregivers. Despite the completion of home settings and family education, children on HIMV may encounter various respiratory problems during home follow-up. Prevention and timely management of these complications are crucial to improving patient outcomes. This article summarizes the most significant respiratory problems in children on HIMV and the management strategies for each problem are discussed, emphasizing the importance of appropriate aspiration techniques, regular monitoring, adequate training of caregivers, and a well-prepared emergency plan.