Recently, mantle cell lymphoma (MCL) has classificated as non Hodgkin's lymphoma. This tumour is a rare type of lymphoma (5-10% of all malignant lymphomas). We reported this case with primary affectation of lacrimal gland. The patient asked for bilateral ptosis, more evident in the right eye. The lacrimal gland was enlarged and easily palpated in the superior lateral aspect of the orbit. The radiology study confirmed lacrimal gland enlargement with out bony changes. The biopsy and immunohistochemical studies diagnosed MCL and was placed for the haematopathologist in stage IVa. The treatment was based in chemotherapy but this subtype of lymphoma is considered to be aggressive and some times incurable with conventional chemotherapy. The median survival is considered to be 3 years.