Lhermitte-Duclos disease or dysplastic gangliocytoma of the cerebellum is a rare benign unilateral mass of the cerebellar cortex having unique diagnostic MRI features characterized by classic ‘tiger striped’ appearance. Correct recognition of the disease is particularly important given the possible correlation with cowden syndrome and therefore the need to identify concurrent malignancies. MRI is a considered diagnostic and is helpful in distinguishing LDD due to its unique imaging features. Two biopsy proven cases of this unusual disease were reported with atypical presentations. Atypical presentations include- presence of diffusion restriction in the dysplastic left cerebellar hemisphere in one case and involvement of bilateral cerebellar hemisphere and vermis in the second one.
