Recent advances in pediatric medicine have led to increasing numbers of severely disabled children who require medical care at home. Severely disabled children were defined as those who have or are at increased risk of having a chronic physical and developmental condition. On April 1, 2015, we conducted a survey of these children aged 18 years or younger living in the Saku region of Nagano Prefecture. Forty severely disabled children were identified in the Saku region, accounting for 0.02% of the population. Thirty children were being cared for at home and 10 had been institutionalized. The underlying illness or disorder occurred in the perinatal period in 21 children (52.5%). We further investigated the medical care and social support of those severely disabled children living at home. There were 8 children with an extremely severe disability and 7 with a moderately severe disability. Fifteen children did not fall into either category. One patient regularly received medical examination and care via visiting serv­ices and the remaining patients received outpatient care. Visiting nurses provided support and care for 10 children (33%), especially those who needed medical care, such as for tracheostomy and gastrostomy. Thirteen children (43.3%) used a short-stay service, and 19 children (63%) used outpatient support (after-school daycare service/day care service for holidays). Short-stay facilities and child day care services are lacking. The custody support system needs to be enhanced in this region for children and their family members to lead healthy lives.