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Leukemia is the most common malignancy of child-hood representing about 30 % of oncohematological diseases diagnosed in children less than 15 years of age. We report the case of a 4 year old girl with acute promyelocytic leukemia whose blasts showed the morphology characteristic of acute promyelocytic leukemia variant. The case is reported because in the paediatric population the acute promyelocytic leuke-mia is a rare occurrence moreover, it represent a true oncohematology emergency, in this case the labora-tory has a significant role since the timing of diagnosis must be very short. It helps in therapeutic protocols compared to conventional therapeutic protocols in Acute Myeloid Leukemia (AML), the introduction of retinoid All-Trans-Retinoic Acid (ATRA), both in children and adults with Acute Promyelocytic Leuke-mia (APL), has significantly reduced the early mortal-ity