Introduction: Primary deposition of amyloid can take several forms including amyloidoma organ restricted among which is brain. Brain amyloidoma can mimic several lesions and offers a diagnostic challenge to the physician. The present work reports an additional case and summarizes the salient characteristics of previously published cases. Case Report: A 61-year-old, woman experienced paroxystic auditory hallucinations followed at times by generalized tonico-clonic seizures. Examination revealed a mild left pyramidal weakness. Head CT scan and MRI revealed a lobular lesion of the right atrium with contrast enhancement. The diagnosis of amyloidoma Lambda light chain (λLC) type was performed by means of a stereotactic biopsy. The patient was treated by 3 cures of high dose methotrexate (15 mg/m 2 ). At 24 months follow up the patient remains stable clinically and biologically. The 2 years follow-up MRI showed the same findings. Conclusion: No guidelines can be suggested for brain amyloidoma treatment; the "wait and see" was the mind-set in the literature. However, a non-tumour like pattern of a cerebral mass developing near the ventricle with a fine, irregular radiating margin enhancement and lack of oedema, and mass effect are important MRI clues for brain amyloidoma diagnosis. The diagnosis is histological after biopsy or excision.