Background Benign tremulous parkinsonism (BTP) is a tremor dominant syndrome characterized by mild, levodopa-resistant parkinsonism with limited disability or progression. Case Presentation A 56-year-old woman presented with a 2-year history of tremor. Neurological examination revealed right-hand rest tremor and slow finger tapping with decreased amplitude; however, we did not observe posture tremor, rigidity, bradykinesia, or posture disability. She was diagnosed with Parkinson's disease (PD) and received levodopa/carbidopa, effectively treating her rest tremor. At the age of 61 years, reoccurrence of the rest tremor was successfully treated again with levodopa/carbidopa and selegiline. Approximately 11 years have passed since symptom onset and the patient shows no further disease progression. Conclusion This case broadens the characterization of BTP to include levodopa-responsive PD.