Primary cutaneous involvement in B-cell lymphoblastic leukemia is rare in childhood. We present a case of 2-year-old girl admitted to our hospital because of left scapular skin lesion and left axillary mass. She was looked pale and weak. Left axillary lymph node pachage of 5 cm diameter and left scapular skin lesion was revealed by physical examination. Complete blood count revealed 124.000/mm3 white blood cell count. Peripheral blood smear showed 99% lymphoblasts. Bone marrow aspiration revealed 95% blastic cells with immunophenotype and morphological characteristics of pre-B type acute lymphoblastic leukemia with L1 subtype. ALL St Jude Total XIII remission induction treatment protocol was started. Skin lesion disappeared after 15th day of the cytotoxic therapy. On the follow up, she was on remission and continued to maintenance chemotheraphy for 6 months. We would like to highlight that a small growing cutaneous lesion could be the presenting form of acute lymphoblastic leukemia.