RESUMO
Ocular adnexal lymphoid tumors are slow-growing neoplasms that affect predominantly the elderly. The purpose of this study is to report a non-Hodgkin lymphoma from mantle zone cells, a rare ocular annexal B-cell lymphoma subtype. CASE REPORT: A 62-year-old male patient with superior eyelid, maxillary and oral tumours, had an initial diagnosis of Mikulics disease, developed lesion enlargement, despite immunosuppressant therapy. Left superior eyelid and bone marrow biopsy revealed non-Hodgkin lymphoma the mantle zone. In spite of dissemination (stage IV), chemotherapy and bone marrow transplant led to disease remission. CONCLUSIVE COMMENTARIES: Despite the availability of advanced complementary diagnostic methods, like immunophenotyping and molecular genetic analysis, lymphoma represents, for physicians and pathologists, a challenge regarding diagnosis and prognosis.