Lhermitte-Duclos disease (LDD) is a rare, slow-growing, benign lesion of the cerebellum. It is often seen in the second and fourth decades. This disease is extremely rare in childhood. A 6-year-old girl presented with loss of balance. A mass lesion in the right cerebellum was detected by magnetic resonance imaging. The patient underwent surgical removal of the lesion, and the histological diagnosis was dysplastic gangliocytoma (LDD). The patient was discharged without complication, and her balance improved in the follow-up period. In this report, we present this rare occurrence in childhood and discuss the clinical course and management. LDD is very rare in early childhood and should be considered in the differential diagnosis of posterior fossa lesions.
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