Angioleiomyoma is an exceptional, benign, solitary neoplasm originating from tunica media of vascular smooth muscle. The neoplasm commonly represents as a uniform, well defined, solitary, soft, nodular, gradually progressive, bluish or purple lesion commonly beneath <2 centimetres magnitude with an absence of accompanying inflammation. Tumefaction is composed of thickened smooth muscle fibres and vascular channels with circumscribing attenuated fibrous tissue capsule. Additionally, the lesion may denominate organizing thrombus, mature adipose tissue cell aggregates and lymphocytic infiltrate. The neoplasm is immune reactive to desmin, myosin, α-smooth muscle actin, HHF35, calponin and h-caldesmon. Angioleiomyoma necessitates a segregation from angiomyolipoma, leiomyoma with bizarre nuclei, glioblastoma multiforme, arteriovenous malformation, cavernous haemangioma, angiofibroma, primary intracranial myopericytoma, glomus tumour, traumatic neuroma, eccrine spiradenoma, angiolipoma, ganglion cyst, neuroma, fibroma, lipoma, neurofibroma, neurilemmoma, salivary gland neoplasms as mucocoele, pleomorphic adenoma, vascular neoplasms as lymphangioma, haemangioma, pyogenic granuloma or dermoid cyst. Comprehensive surgical excision of the lesion with a tumour -free perimeter is an optimal treatment strategy.