Orbital lymphoma is a rare condition with non-specific and variable clinical course. This features are the main obstacle for diagnosis and treatment. A 43-year-old female patient complaining of vision loss and hemiface paresthesia, Proptosis was noted associated with an expansive lesion involving the right temporal lobe and orbital region. Craniotomy was performed to allow orbital decompression and anatomopathogical analysis. Final diagnosis was Burkitt Lymphoma. Patient evolved to death after seven days. A 40-year-old male patient complaining excessive tearing and sight disturbance. Proptosis on the right eye was noticed. Extra-conal expansive lesion was observed. Final diagnosis was mucosa-associated lymphoid tissue lymphoma. Radio and chemotherapy protocol was initiated. In a 3 year follow-up, there are no signs of recurrence or ocular/orbital impairment. Maxillofacial surgeons should be aware of the initial clinical presentation and always include lymphoid neoplasms as a differential diagnosis in cases which acute functional alterations are present.
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