Ichthyosis is a hereditary dyskeratotic skin disease with systemic skin dryness and roughness, mainly manifested by scaly skin, which may be accompanied by ocular abnormalities. At present, there are many studies on skin fungal infection caused by ichthyosis, but only few reports on cases with combined ocular fungal infection. This paper reports a case of X-linked recessive hereditary ichthyosis with recurrent fungal keratitis (FK), which is expected to provide reference for clinical early diagnosis and treatment of this disease.