Objectives: Aggressive angiomyxoma is known as a mesenchymal tumor of premenopausal women and it is extremely rare in men. Methods: Herein, we report a 66-year-old male with a firm scrotal mass that had gradually enlarged over 20 years. Results: Radiological studies revealed 10 x 15 cm mass lesion confined to right scrotum with neither local invasion nor distant metastasis. Inguinal orchiectomy was performed and histopathology showed characteristic features of an aggressive angiomyxoma occupying paratesticular region, which was a challenging diagnosis due to its unexpected occurrence in the male gender. Conclusion: Aggressive angiomyxoma is mostly considered as a benign tumor in females despite its propensity for local recurrence. Whether it may show a divergent biological behavior in men is unknown as the reported cases are too few.