This case report represents a child that needed, at the age of 3, dental treatment under narcosis due to nursing bottle caries including multiple extractions. Six months later the few remaining teeth were again seriously affected by decay. Due to the medical history of urological problems, abnormal aspect of hair and skin and abnormal forms of primary teeth (only reported by the parents), there was a presumption of ectodermal dysplasia, so the patient was referred to the department of medical genetics. Genetic research is still going on. We decided to restore the remaining teeth in ambulant care with plastic restorations and stainless steel crowns. On demand of the parents, we decided to provide the child with a removable denture to increase his self-confidence. The child has now reached the age of 6 and has well accepted the removable denture. These served furthermore as an appliance to maintain space and prevent tooth migration. Removable dentures in young children can be a successful intermediate solution by lack of primary teeth.