To present the case of a 57-year-old male with progressive bilateral proptosis. The patient presented with bilateral proptosis and strength deficiency on the upper limbs. During hospitalization, the progression was unfavorable; proptosis progressed, causing a severe loss of vision in the left eye (from 0.8 Snellen to NLP). The imagistic investigation revealed bilateral infiltration of the orbits, infiltrative lesions to the mediastinum and the abdomen. The patient was referred to neurosurgery for further management. Surgical orbital decompression was performed with biopsy. The histopathological examination revealed non-Hodgkin small cell lymphoma. Assembling the clinical and paraclinical data we have suspected the possible diagnosis of Erdheim-Chester disease, however, positive diagnosis has not been achieved.
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