Leukoencephalopathy with intracranial calcifications and cysts (LCC) is a rare and recently described entity characterized radiologically by white matter abnormalities, calcifications, cysts and enhancing nodules 1 . LCC is a disorder of unknown etiology and its neurological manifestations include cognitive decline, seizure, obstructive hydrocephalus, progressive cerebellar, besides extrapyramidal and pyramidal signs with juvenile onset 2 . We report a case of LCC with remarkable radiological and clinical features. CASEA 30-year-old man presenting frontooccipital headache for the last 7 days, worsening in the morning, associated with progressive anorexia and vomiting.Eight years ago, he had an acute neurological event characterized by paresis and aphasia of expression.His past medical history demonstrated moderate cognitive impairment since childhood.Family history, were unremarkable and no consanguinity was known. Neurological examination revealed left paresis grade 4+ with positive Babinski sign besides ataxia. Brain MRI (1.5T) revealed an expansive solid / cystic interhemispheric lesion measuring 55×25 mm with important mass effect. T2 imaging showed remarkable hemossiderin deposition in its walls. MR perfusion demonstrated high capilar density within the walls. Multiple enhancing nodular lesions were observed envolving supra and infratentorial brain parenchyma. Some of these nodules had hemorrhagic component on T2 images. Broad and symmetric calcifications in periventricular white matter, basal ganglia, brainstem and dentate nuclei were observed.Patient underwent surgery for relieving intracranial hypertension and biopsy. Pathological examination was inconclusive due to the large amount of clotted blood within the material. No neoplastic cells were observed. Patient was discharged in good clinical condition, without signs of intracranial hypertension, remaining hemiparesis and aphasia.

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