Congenital icthyosis is a rare group of disorders of keratinisation. A case of this condition is reported in order to highlight the clinical features and essential components of management: resuscitation, skincare, nutrition and counseling. This case is reported in order to appraise clinicians of its presence because there was delay in the diagnosis and management due to non-recognition. Keywords: Ichthyosis, Congenital, Kaduna, Nigeria. Résumé Les démengeaisons congénitales – ichthyosis sont des rares groupes de mutation de la peau-keratinisation. Un cas de cette maladie rare nous ai reporté afin d'éclaisir les differents apparences et leurs traitment: qui inclus la reanimation, les soin de la peau, la nutrition et un exposé aux parents. Ce cas entraine un delai de diagnostics et de traitments du a la non identification de la maladie. Donc, nous tenons a vous rappeler son existence. West African Journal of Medicine Vol.21(3) 2002: 250-251
