Objective: To report a case of congenital pelvic arteriovenous malformation (pAVM) with urinary retention. Clinical Presentation and Intervention: A 39-year-old male patient presented with inability to void urine for 8 h. He did not have a history of dysuria or fever, but had increasing urinary frequency and difficulty in voiding over 2 years. Examination revealed a suprapubic mass without external signs of vascular anomaly. A huge, soft, irregular, non-pulsating mass was felt rectally. This mass did not disappear on urinary bladder decompression. Subsequent ultrasound and CT scan revealed pAVMs filling most of the pelvic cavity. Conclusion: To our knowledge, this is the first report of a male patient with congenital pAVM presenting with urinary retention, emphasizing the need for a high index of suspicion for pAVM to avoid potential severe bleeding during suprapubic catheterization for urinary drainage.