Introduction: Ectodermal dysplasia (ED) is a rare hereditary disorder involving two or more of the ectodermal structures. Palmoplantar keratoderma is a characteristic feature of hidrotic forms of ED. We report a case of Hypohidrotic ectodermal dysplasia associated with palmoplantar keratoderma. Case Report: A 4-year-old girl presents a chronic palmo-plantar dermatosis that started three years ago. The interrogation finds a history of hospital admission in the 2nd month of life for an unexplained fever. The dermatological examination finds a limited, diffuse erythematous palmo-plantar hyperkeratosis, associated with diffuse alopecia with light-colored brittle and slow-growing hair. The findings also include hypotrichosis and onychodystrophy. The rest of the examination revealed a facial dysmorphism with a light prominent forehead, flattened bridge of the nose, sunken eyes with periorbital hyperpigmentation and photophobia. Other abnormalities include a hypodontia with small teeth. Discussion: Our observation suggests a hypohidrotic ectodermal dysplasia. It is a genetic disease most often hereditary. The X-linked form or Christ Siemens Touraine syndrome is the most frequently encountered form. A male predominance is noted. Clinically it is characterized by the presence of all or several of the four typical clinical signs of the disease: Anhidrosis or hypohidrosis, dental hyoplasia, hypotrichosis, facial dysmorphism. Palmoplantar keratoderma is rarely associated as it is the case in our patient. The genetic study reveals abnormalities that can be found in the X chromosome for the X-linked forms, and at the level of the chromosomes 2q11-13 or 1q42 for the autosomal forms. Conclusion: Our observation illustrates the frequent diagnosis delay in this disease. It also emphasizes the importance of a detailed somatic examination in front of any palmoplantar keratoderma presented in children.