Acute lymphoblastic leukemia is the most frequent cancer in children: it represents 80% of leukemias and about 24% of all neoplasms diagnosed between 0 and 14 years. Acute lymphoblastic leukemia mainly affects children between 2 and 5 years old and in this age group the incidence is about 80–90 cases per million per year. In acute lymphoblastic leukemia, cancer cells multiply rapidly and accumulate in the bone marrow and subsequently invade the blood. However, at the time of diagnosis, leukemia rarely occurs outside the bone marrow or blood vessels and the extramedullary involvement happens mostly in patients with refractory or relapsing disease. In this article, we report an unusual clinical presentation of acute B cell lymphoblastic leukemia with intestinal and ovarian localizations in a 5-year-old girl.