A 43-year-old male presented with recurrent Lhermitte-Duclos disease (LDD), a rare pathological entity of the cerebellum of which the etiology is still controversial. He had undergone subtotal removal of a cerebellar lesion, misdiagnosed as a benign astrocytoma, 8 years previously. Subtotal removal of the recurrent tumor completely resolved the presenting symptoms. Recurrence of LDD is not as rare as generally assumed. Patients with LDD require long-term observation even when the initial treatment appeared curative.