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Abstract Lhermitte-Duclos disease (LDD) is a relatively uncommon condition of the cerebellum. It is generally characterised as a hamartomatous lesion of posterior fossa and is common in the third and fourth decades of life. According to the World Health Organisation, it is classified as a grade I tumour with potential for recurrence. Otherwise, this disease is generally associated with good prognosis. Malignant transformation of LDD has not yet been reported. However, genetic counselling of the patient is recommended with active surveillance. Since LDD is believed to be a pathognomonic feature of Cowden syndrome, which is a multi-system autosomal dominant hereditary disorder characterised by multiple hamartomas and an elevated risk of benign and malignant neoplasms, we decided to report this important entity considering its rarity and high clinical significance. Keywords: Lhermitte-Duclos disease, Cowden syndrome, dysplastic cerebellar gangliocytoma, PTEN