STUDY OBJECTIVES: Clinical utility of home polysomnography in children with neuromuscular disorders is limited by lack of evidence that sleep-disordered breathing can be reliably identified and inability to diagnose hypoventilation as carbon dioxide is not measured. METHODS: This study aimed to determine feasibility, accuracy and parent satisfaction for home polysomnography performed with a Type 2 portable monitoring device and a transcutaneous CO monitor. Results of laboratory and home polysomnography were compared with Mann-Whitney U tests and random intercept regression models. RESULTS: Nineteen paired polysomnograms were performed for participants with various neuromuscular disorders at a median age of nine years. Feasibility was 68%, defined as adequate data recorded from the portable monitoring device and transcutaneous CO monitor. Adequate transcutaneous CO data was recorded in 89% of home studies. Significant differences were higher total sleep time, less awake time and less N1 sleep during home polysomnography. Home polysomnography had sensitivity of 50% for diagnosis of hypoventilation and 78% for obstructive sleep apnea. Home polysomnography under-estimated severity of obstructive sleep apnea in three cases. Parent satisfaction for home polysomnography was high. CONCLUSIONS: This is the first study to measure transcutaneous CO during home polysomnography in children with neuromuscular disorders. Results suggest better sleep quality at home during testing. Current sensitivity of home polysomnography for the diagnosis of sleep-disordered breathing is too low for clinical use, however, the feasibility of transcutaneous CO measurement in the home and high satisfaction justifies further research to improve accuracy of home polysomnography in this population.