A nine-year-old girl was presented to the dental clinic at King Abdulaziz Medical City in Jeddah with carious posterior primary teeth and discolored teeth. She was referred to a pediatric geneticist to rule out any medical conditions. She was suspected to have ectodermal dysplasia (ED). DNA testing was requested and sent to Germany to confirm the clinical findings. Afterward, full-mouth rehabilitation was done including prophy and fluoride application, composite restorations, stainless steel crowns, and pulp therapy. The treatment was planned to be carried out under local anesthesia due to the cooperative behavior of the child and the parents’ request. Treatment objectives were oriented to achieve esthetic and functional rehabilitation of all carious teeth, maintain occlusion, in addition to maintaining spaces for the missing permanent teeth for future replacement. The patient became caries-free. Oral hygiene was improved which resulted in gingival inflammation resolution. Spaces were maintained and the behavior of the child was improved during recall visits from positive to definitely positive. Multidisciplinary approach in the management of oral and dental manifestations associated with ED including pediatric dentists, prosthodontists, and orthodontists is of paramount importance.