INTRODUCTION: Aggressive angiomyxoma (AA) is a rare mesenchymal tumour of the connective tissue of the pelvis, which was described mainly in women in their reproductive period of life. Until now 45 cases of AA in men are documented with predominantly inguinal, parafunicular or scrotal localisation. These tumours slowly infiltrate the adjacent tissue and since symptoms are noticed only later these tumours have reached a considerable size at the time of diagnosis. In contrast to their benign histological appearance and almost entire absence of metastasis AA tends to (multiple) relapse. CASE REPORT: We report on a 46-year-old male with a large tumour in the perineum. After complete resection, histological analysis revealed an AA. 26 months after surgery there is no evidence of relapse. CONCLUSION: AA has to be considered as a possible diagnosis for obscure tumours of the pelvis. Since these tumours tend to relapse, margin-negative resection is most important. Also in cases of relapse, secondary excision should be achieved within healthy tissue. Adjuvant hormonal chemotherapy or radiation can be considered in cases of multiple relapse. Because of the risk of local recurrence rather than metastasis, these patients need a long-term follow up.
