Although primary hemifacial spasm (HFS) is mostly related to a vascular compression of the facial nerve at its root exit zone (REZ), its occurrence in association with distal, cisternal portion has been repeatedly reported during the last two decades. We report two patients with typical HFS caused by distal neurovascular compression, in which the spasm was successfully treated with microvascular decompression (MVD). Vascular compression of distal, cisternal portion of the facial nerve was identified preoperatively in the magnetic resonance imaging (MRI). It was confirmed again with intraoperative findings of compression of cisternal portion of the facial nerve by the meatal loop of the anterior inferior cerebellar artery (AICA) and absence of any offending vessel in the REZ of the facial nerve. Immediate disappearance of lateral spread response (LSR) after decompression and resolution of spasm after the operation again validated that HFS in the current patients originated from the vascular compression of distal, cisternal portion of the facial nerves. According to our literature review of 64 patients with HFS caused by distal neurovascular compression, distal compression can be classified by pure distal neurovascular compression (31 cases, 48.4%) and double compression (both distal segment and the REZ of the facial nerves, 33 cases [51.6%]) according to the presence or absence of simultaneous offender in the REZ. Eighty-four percent of 64 identified distal offenders were the AICA, especially its meatal and postmeatal segments. Before awareness of distal neurovascular compression causing HFS and sophisticated MRI imaging (before 2000), the rate of reoperation was high (58%). Preoperative MRI and intraoperative monitoring of LSR seems to be an essential element in determination of real offending vessel in MVD caused by distal offender.