Erythrokeratodermia variabilis is a rare genodermatosis associated with keratinisation disorders. We report a case of erythrokeratodermia variabilis in Moroccan infant. A 22-month-old girl was referred to our department with reddishbrown hyperkeratotic and well-demarcated plaques on the extremities. The lesions started when he was 4 months of age. The patient was treated by emollients and keratolytic cream. Erythrokeratodermia variabilis is characterized by the association of fixed well-demarcated plaques and transient erythematous patches. Although cutaneous histopathology is not specific, a typical physical clinical feature can aid the diagnosis.