Objective With advances in technology, pediatric patients are sent home with chronic invasive mechanical ventilation (CIMV). Ventilation optimization and preparation for weaning are essential processes, however, there is no standard approach to weaning and titrating ventilator settings in children. There is little information in the literature on the utility of polysomnography (PSG) in weaning and titration of CIMV. Our objective is to review the role of PSG on titration and weaning of CIMV in children. Methods A retrospective review of medical records and polysomnograms was performed on CIMV dependent patients at Cincinnati Children's Hospital Medical Center from January 2011 to October 2017. Patients underwent overnight ventilator weaning or titration PSG. Subjects with less than 3 h of total sleep time were excluded. Results About 163 PSGs were divided into 97 PSGs for ventilator titration and 66 PSGs for ventilator weaning. Of the 97 ventilator titration PSGs, 59 (60.8%) had inadequate ventilation, 10 (10.3%) had inadequate oxygenation, 22 (22.7%) had significant tracheostomy leak, 13 (13.4%) had autocycling, 3 (3.1%) had failure to trigger, 4 (4.1%) had central sleep apnea, and 24 (25.8%) had adequate ventilation on current ventilator settings. Of the 66 ventilator weaning PSGs, 48 (72.7%) weaned to lower ventilator settings or off ventilator support and 18 (27.3%) did not wean. Discussion Our results indicate that PSG is useful in titration and weaning of ventilator settings in children with CIMV. Larger studies are needed to evaluate the subgroup of this population who would benefit most from PSG for assessment of adequate ventilator support.