Introduction. Lhermitte—Duclos disease is a rare autosomal dominant inherited disorder characterized by the loss of the normal cerebellar cortex architecture and hamartoma formation in the cerebellar hemispheres. Most commonly, this disease manifests in the third and fourth decades of life. Approximately 220 cases of Lhermitte—Duclos disease have been reported in medical literature to date. Material and methods. The authors describe successful two-stage surgical treatment of a young female patient with Lhermitte—Ducos disease. Conclusion. This case report familiarizes practitioners with the clinical manifestations and neuroimaging features of Lhermitte—Duclos disease that facilitates timely diagnosis and proper treatment of the condition.