Ectodermal Dysplasia is a group of closely related conditions involving at least two of the ectoderm structures (hair follicles, sweat glands, teeth, nails, and salivary glands). Oral findings are significant and usually multiple abnormalities affect both function and aesthetics. This plays a major role in the psychological development of the child as the condition manifests itself at an early age. The present case describes an 8-year-old girl who showed the characteristic features of Hypohidrotic Ectodermal Dysplasia (HED) and a multifaceted approach to the dental management of the patient.