The authors report a 9-year-old boy presenting with a left cerebral ischemic infarction as the first manifestation of acute promyelocytic leukemia. During consolidation chemotherapy, the patient developed nephrotic syndrome and a renal biopsy revealed focal segmental glomerulosclerosis (FSGS). Remission in bone marrow was achieved with chemotherapy, however, new intracranial ischemic areas developed on follow-up. Acute promyelocytic leukemia complicated by FSGS has not been previously reported in children. There may be a relationship between anthracycline treatment and FSGS. Thrombosis could be related with both leukemia and nephrotic syndrome, here thrombosis was the initial symptom, before FSGS was diagnosed.