Lhermitte–Duclos disease (LDD) is a rare cerebellar lesion of uncertain origin. It is linked to an autosomal– dominant phakomatosis known as Cowden’s disease in 40% of patients. The MRI features of LDD are almost unique and can be considered diagnostic. (1) We report on a  case of  48 year old female patient who presented to us with history of seizures and bilateral lower limb weakness with the typical MRI features of the above disease. We also discuss the pathology and genetics of this rare disease.