Angioleiomyomas (ALMs) involving the central nervous system are exceedingly rare, and no ALM involving the parafalx region has ever been clinically reported. We report the first case of ALM involving the parafalx region on its surgical procedure and radiological feature. A 50-year-old man had a 6 month history of frontal headache and occasional seizure attacks. The radiological examination showed a parafalx occupation similar to meningioma. The tumour was very hypointense on T1 weighted magnetic resonance imaging (MRI) and hyperintense on T2 weighted MRI, which enhanced homogeneously with intravenous administration of gadolinium. The tumour was removed totally by sinusoidal transverse scalp incision and bilateral parafalx approach. To our surprise, the tumour was prominently debulked using bipolar shrinkage to coagulate the tumour feeding vessels, resulting in less bleeding during total removal of the tumour.. The lesion was confirmed as ALM by histological examination. The prognosis was good for the patient after surgery during the 18 month follow-up.