Non-Hodgkin lymphoma is rare in children, even though it is the third most frequent type of tumour. Management of a child with non- Hodgkin lymphoma is complex and coordinate presence of haematologists, surgeons, radiotherapists, neurologists, psychologists and other expert personnel is required. Our patient presented with a bulky mass of approximately 5 cm in diameter, which grew from his upper-left maxillary bone, thereby causing gum bulge. Thus, the left side of his face from lip to eye appeared swollen. A whole body computed tomography- positron emission tomography examination revealed that the mass was growing in the mouth and maxillary bone and that many bulky nodes were present both in the right and left neck. Histological haematoxylin and eosin assessment revealed an anaplastic large cell proliferation underlying the epithelial tissue without epithelial infiltration. The patient underwent a first cycle of chemotherapy according to the 'International Protocol of anaplastic large cell lymphoma'. The Maxillofacial Surgery Unit and the Dentistry Unit of the same hospital took care of his dental situation to avoid the spread of infective foci to the entire body. After 1 year and 3 months from the first cycle of chemotherapy, a bulky splenic mass was discovered. Laparoscopic biopsy revealed a relapse of the anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a splenic node. The patient is now alive in good conditions, and continuously followed-up by the Pediatric and Hemato-oncology Operative Unit of University Hospital of Parma. Rapidity of action and a correct multidisciplinary (oncology- maxillofacial surgery-dentistry) approach is the key to cure illness, promptly diagnose relapse and avoid the spread of infective foci from the patient's teeth during chemotherapy cycles.
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