INTRODUCTION
Ectodermal dysplasias (ED) are a heterogeneous group of disorders resulting from abnormalities of structures derived from embryonic ectoderm. Rapp-Hodgkin syndrome (RHS) was first described over 30 years ago in an affected mother, son, and daughter with a combination of ED, cleft lip and cleft palate (1). Other features include narrow nose, thin coarse hairs, hypodontia with hypoplastic enamel and malformed teeth, hyponychia, anonychia, narrow or dystrophic nails, lacrimal duct abnormalities, ear and ear canal abnormalities (2). Here, we describe a 15-year-old male who has RHS associated with palmoplantar keratoderma.