The combination of vestibular schwannoma (VS) and hemifacial spasm (HFS) caused by a vascular loop is not frequently encountered. Surgical intervention using the retrosigmoid approach may enable the two diseases to be treated simultaneously. A 68-year-old male presented with progressive right HFS. MRI revealed a concurrent vascular loop and 3.2-mm intracanalicular VS. The retrosigmoid approach, together with microvascular decompression, was used to remove VS. Intraoperatively, compression by the vascular loop alone induced HFS although it was not related to VS. This finding is in contrast to the literature reports in which VS has been implicated in HFS. In our patient, when VS was completely removed, HFS immediately disappeared postoperatively.