Abstract We report a case of a male patient with an aggressive angiomyxoma located in the presacral space which was diagnosed and clinically treated at the Hospital Militar Central in Bogota, Colombia. Kraske’s posterior appro-ach was used to completely excise the lesion with minimal functional and aesthetic impact on the patient. Aggressive angiomyxomas are extremely rare. They are most commonly found in pelvic locations in women. They are even more exotic in men, with very few cases reported in the literature. Management requires com-plete removal of the lesion and the clinical and imaging follow-up given their high rate of recurrence. Key words Aggressive angiomyxoma, presacral tumor, Kraske’s approach. INTRODUCTION Aggressive angiomyxoma (AA) is a rare pelvic soft -tissue tumor oft en found in the perineum which originates from myocardial fi broblasts. It is locally aggressive and metasta-sizes quickly and easily. First described in 1983 by Steeper and Rosai in the soft tissues of the pelvis and perineum of premenopausal women, these tumors appear to be benign in cytology, composed of spindle and star cells embedded in a myxoid matrix containing medium caliber vessels. Nevertheless, despite their cytologically harmless appea-rance, they are infi ltrating tumors with high risks of recu-rrence (1, 3, 5-7). Because they are rare, their incidence has not been clearly established (1), but about 190 cases have been described in the medical literature to date. Of these approximately 150 have been reported in premenopausal women from 30 to 40 years of age and 40 cases have been reported in men (4, 5, 7, 12). Of these cases only about 70 have had been located in the pelvis or in retroperitoneal locations (3). It has been established that fewer than 150 of the cases were reported in the English language literature between 1983 and 2004 (2). In 2007 fi ft een additional cases in were described men in the English and fi ve more in Spanish-language literature (1). Incidence and reports of the disease have been increa-sing each year (12). Th e entity occurs at least 7 times more oft en among women as it does among men. It has been reported in male and female children as young as 2 years old (2, 3, 5). It is most frequently found in the scrotum, perineum, groin, pelvis and spermatic cord (1). In women it is most com-monly found in the perineum and pelvic organs including the bladder and uterus (3).