A 5-year-old Chinese boy presented with difficulty in walking and weakness of his lower limbs for one year, especially towards the evening. Bilateral equinovarus posturing of the feet and tremors of the upper limbs were noted on physical examination. Dopa-responsive dystonia was diagnosed after a remarkable symptomatic response to levodopa. This disorder is reported here to highlight an often misdiagnosed condition is children which is important because it is treatable. Dopa-responsive dystonia should be considered in the differential diagnosis of gait disturbance in children.