Aggressive angiomyxoma of the vulva is uncommon and the pathogenesis remains unclear. Recently, a few cytogenetic studies demonstrated chromosomal abnormalities in aggressive angiomyxoma of the vulva. A 45-year-old Japanese woman complaining of right-side vulva tumor measuring 3 cm in diameter was referred to our hospital. The mass was located at the right side of the rectovaginal septum and was closely adherent to the rectal serosa and the levator muscle ani. The tumor was completely resected. A pathological examination demonstrated aggressive angiomyxoma of the vulva. Chromosomal analysis of the tumor demonstrated a sole t(5;8)(p15;q22) translocation. This is the first case of an aggressive angiomyxoma of the vulva with a sole t(5;8)(p15;q22) translocation.