Introduction: Acute lymphoblastic leukemia (ALL) is the most common hematologic malignancy in children. The clinical presentation at diagnosis is due to bone marrow infiltration or extra-medullary involvement. Maxillofacial localization is very rare in ALL. We report a case of T-ALL revealed by right cheek swelling in a 14-year-old boy., Case Presentation: A 14-year-old boy had presented with a 6 cm right cheek swelling for two months, complicated by pyrexia, cervical adenopathy, and splenomegaly. CT scan revealed a 3×7×8 cm swelling surrounded by voluminous sub chin, subclavicular, mediastinal, and paratracheal bilateral adenopathies. The biological analyses revealed normocytic anemia, hyperleukocytosis with 83.6% blast cells, neutropenia, and thrombopenia. The biopsy histology and the immuno-histochemical analysis suggested a diffuse small-cell Non-Hodgkin lymphoma (NHL). The myelogram identified a type 1 ALL and immunophenotyping on bone marrow cells suggested phenotype T ALL. The patient was treated according to the MARALL-06 protocol and died on the 17th day of induction, in septic shock., Discussion: Despite their rare occurrence, ALL should be included in the differential diagnosis of jugal swelling. The evident hematological context should suggest the diagnosis and a myelogram should be performed as soon as possible because the outcome is rapidly fatal., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)