• Gautam Dutta, Daljit Singh, Ravindra Kumar Saran, Hukum Singh, Arvind Kumar Srivastava, and Anita Jagetia.
• Department of Neuro-Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India. Electronic address: gautamblue@hotmail.com.
• World Neurosurg. 2018 Sep 1; 117: 344-349.

BackgroundLhermitte-Duclos disease is an extremely rare pathologic entity characterized by a cerebellar mass composed of enlarged cerebellar folia containing abnormal ganglion cells. This entity usually presents in young and middle-aged adults and rarely in children. There is no study in the literature analyzing the long-term clinical course of this disease to assess the behavior primarily because of its rarity.Case DescriptionWe present our experience with a 7-year-old patient of Lhermitte-Duclos disease who was followed up for 5 years and found to have progressed to bilateral World Health Organization grade IV medulloblastoma. This case denotes the malignant potential of this rare disorder.ConclusionsLDD is seen rarely and demands a high degree of suspicion in patients presenting with cerebellar mass and/or imaging characteristics. It is prudent to keep these patients in close follow-up for early detection of malignant transformation.Copyright © 2018 Elsevier Inc. All rights reserved.

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