Background Lhermitte-Duclos disease is typified by a hamartomatous lesion of the cerebellum. It is usually seen in females. The usual presentation is of raised intracranial pressure along with cerebellar signs. Case report We report a 18 year female patient who presented to us with history of headache and gait ataxia and on imaging was observed to have Lhermitte-Duclos disease with cervical cord syrinx also. Discussion To our knowledge this is the fifth case of Lhermitte-Duclos disease with syringomyelia in pediatric age group. The treatment of this condition is decompression of the lesion. Malignant transformation never occurs and prognosis is excellent.