Holmes’ tremor is an unusual combination of rest, postural and kinetic tremor of the extremities. Medical treatment of this condition still remains unsatisfactory. We report the case of a 20-year-old female patient who developed right-sided Holmes’ tremor nine months after a left-sided, cavernoma induced midbrain/pontine hemorrhage at the age of 16. Beta-CIT SPECT revealed abolished dopamine transporter activity in the left basal ganglia and striatum, in accordance with missing ipsilateral tegmento-frontal connectivity (medial forebrain bundle) demonstrated by diffusion tensor MRI (DTI). Tractography showed reduced fiber connectivity of the superior and middle cerebellar peduncles on the lesioned side. Administration of pramipexole and L-DOPA led to a clinically significant reduction of tremor severity. In conclusion, our results support the notion of Holmes’ tremor to be the result of diminished striatal dopaminergic input in our patient.