Abstract: 　Objective　To explore clinical characteristics, treatment and prognosis of patients with X-linked recessive ichthyosis (XLRI) and leukemia. Method　Clinical data of a 10 year-old boy with XLRI complicated with acute promyelocytic leukemia (APL) transformed to erythroleukemia was retrospectively analyzed. Results　The patient had XLRI with bilateral cryptorchidism, hypospadias, pulmonary hypospadias, myocardial abnormalities and malnutrition. He was diagnosed as PML-RARa positive APL at 7 years old, then achieved continuous remission with regular chemotherapy, but transferred to erythroleukemia at 10 years old. Conclusions　XLRI is a dermatological disease caused by steroid sulfatase enzyme deficiency. Patients with congenital ichthyosis have higher incidence of leukemia. There is no special treatment strategy for those patients with ichthyosis complicated with leukemia compared to isolated leukemia, but the former often has unfavorable prognosis.