We are describing the case of a seventy-four year old woman presenting a voluminous hard and painless tumor of the right lids, which has appeared half a year ago. Past medical history consists in a Sjögren's syndrome secondary to rheumatoid arthritis. The right eye is not visible and the left eye presents an important xerophthalmia and an opaque and vascularized cornea. CT Scan and biopsy allow to diagnose a diffuse monocytoid B cell non hodgkin-lymphoma, starting from the lacrymal gland with an extension to the lids and the anterior orbit. The appearance of a non hodgkin lymphoma in the course of the evolution of a rheumatoid arthritis and/or Sjögren's syndrome has been described well. The monocytoid feature of this lymphoma with the Sjögren's syndrome has recently been reported. However, the orbital localisation of this lymphoma is rare.
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