Background: Midbrain tremor is a rare and complex condition of the upper extremities, comprising a combination of resting, postural and kinetic tremors. Midbrain tremor is usually seen in the pathologies of the thalamus, upper brainstem and cerebellum. In midbrain tremor cases resistant to medical treatment, ventralis intermedius nucleus thalamotomy, dual-lesion or single-target stimulation have been applied with different success rates. Case Description: In this report, we present the case of a 21-year-old female with midbrain tremor involving atypical features, treated using a pedinculopontine nucleus lesion. Different targets for the treatment of midbrain tremor using radiofrequency lesion have been reported. In this case, stimulation of the thalamic ventralis intermedius nucleus and subthalamic nucleus produced no satisfactory improvement in tremor frequency and amplitude. A third goal was to obtain a significant response in terms of these outcomes with pedinculopontine nucleus stimulation. Conclusion: According to the literature, this is the first case of midbrain tremor treated with a pedinculopontine nucleus radiofrequency thermocoagulation lesion.