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Background: The prevalence of children receiving long-term mechanical ventilation at home (LTMV-H) is rising in many countries as advances in technology and medicine extend the survival of such patients. In Italy, the prevalence is 4.2 per 100,000 pediatric patients. It becomes essential to thoroughly assess these patients’ needs, and of the quality and adequacy of their home care. Pediatric palliative care (PPC) may be an appropriate strategy for the global care and quality of life of children needing LTMV-H and their families. Objective: To characterize children on LTMV-H in the Veneto Region (north-east Italy) based on the experience of the Veneto Regional Center for PPC and Pain Control (VRCPPC). Subjects: Children and adolescents (0-18 years of age) receiving invasive and noninvasive LTMV-H in the care of the VRCPPC from 01/09/2008 to 31/12/2013. Methods: A retrospective cross-sectional analysis conducted by developing a regional database of children on LTMV-H. Results: 56 children were on LTMV-H (mean age 4.5 years, 55% male), during the period considered. At the time of the survey, 38 of them were still in the care of the VRCPPC, 10 had died and 8 had been discharged. The children on LTMV-H accounted for 50% of all patients in the care of the VRCPPC (38/76 patients). They suffered mainly from neuromuscular diseases (NMDs) (17 patients), myopathy (11 pts), congenital central hypoventilation syndrome (CCHS) (7 pts), or nervous system disorders (7 pts). All patients had severe comorbidities. Their mean age when LTMV-H was started was 3.9 years. Ventilation was invasive (IMV) in 31 cases and noninvasive (NIV) in 25. Its initiation was mandatory in 39 cases (and in the intensive care setting for 36 of these children) and elective in 17 (and 15 of these children were in hospice care). Ventilation was provided for a mean 17.5 hours a days (14 for NIV; 18 for IMV). Median 9-year survival was 61%, but varied considerably by type of patient, being longer for NMDs, myopathy, CCHS, nervous system disorders (over 11 years in 78% of cases). Conclusion: This population has complex life-long needs and numerous comorbidities that demand appropriate, continuous and qualified care. PPC could be an efficient strategy for meeting health goals and optimizing treatment planning.