Lymphoma is an aggressive pathology; revelation by sinonasal involvement is rare. Recommended treatment is chemotherapy, possibly associated to radiotherapy. Prognosis depends on histologic type, Ann Arbor stage at diagnosis and the therapeutic options available for the individual patient.

Total resection of all affected nodes and infiltrated subcutaneous fatty tissue is the treatment of choice. Drug therapy (including at least a macrolide) seems indicated only in case of incomplete resection or if surgery would entail functional and/or esthetic risk. Increased incidence, since BCG vaccination was stopped, will continue to confront the practitioner with an infantile disease in which management must be multidisciplinary.

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