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 an 18-year-old female patient who presented to us with history of headache and gait ataxia and was observed to have Lhermitte-Duclos disease with cervical cord syrinx on imaging.

DISCUSSION: To our knowledge this is the fifth case of Lhermitte-Duclos disease with syringomyelia in the pediatric age group. The treatment of this condition is decompression of the lesion. Malignant transformation never occurs and the prognosis is excellent.