Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder that results in arteriovenous malformations (AVMs) in the nose, mucocutaneous surfaces and visceral organs, including lung, brain, liver, bowel and rarely spinal cord. We describe a case of a young child with HHT who presented with acute paraparesis due to acute thrombosis of a spinal perimedullary arteriovenous fistula. Patient underwent coil embolization of spinal arteriovenous shunt with resolution of clinical symptoms. This case highlights the possibility of catastrophic complications in young children with HHT, the potential preventive role of screening for spinal AVMs in HHT and the importance of timely intervention.

遗传性出血性毛细血管扩张症 (HHT) 是一种常染色体显性遗传病，可导致鼻子、粘膜皮肤表面和内脏器官（包括肺、脑、肝、肠和罕见的脊髓）的动静脉畸形 (AVM)。我们描述了一例患有 HHT 的幼儿，由于脊髓周围动静脉瘘的急性血栓形成而出现急性轻瘫。患者接受了脊髓动静脉分流线圈栓塞术，临床症状得到缓解。该病例强调了患有 HHT 的幼儿出现灾难性并发症的可能性、筛查 HHT 中脊柱 AVM 的潜在预防作用以及及时干预的重要性。