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Lhermitte-Duclos disease (LDD) is a rare, slow-growing neoplasm that develops in the brain's posterior fossa. It can appear as a single lesion or as part of Cowden's syndrome. We report the case of a 51-year-old female with a history of diabetes, hypertension, and a previously treated neuroendocrine tumor, who presented to the hospital after experiencing a generalized tonic-clonic seizure. Except for a tongue laceration, the neurological examination was unremarkable. Brain magnetic resonance imaging (MRI) showed a T2 left cerebellar hemisphere pseudomass lesion with iso-hyperintense signals suggestive of Lhermitte-Duclos disease. This case describes a unique presentation of LDD and its various radiological manifestations, emphasizing the importance of neuroimaging in its diagnosis. Additionally, it contributes to the expanding literature on the varied manifestations of LDD., (© 2024 The Authors.)

The dysplastic gangliocytoma of the cerebellum, also known as Lhermitte-Duclos disease, is an uncommon hamartomatous lesion in the posterior fossa with some distinctive neuroradiological characteristics. It can happen in combination with Cowden syndrome or sporadically. Cowden disease, or multiple hamartoma-neoplasia syndrome, is a rare autosomal dominant condition which is characterized by mucocutaneous lesions and systemic malignancies. We present a case of Lhermitte-Duclos disease and Cowden disease occurring in adult patients. The clinical and radiological features as well as the management approaches of this unusual disease complex are addressed., (© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)