肛周坏死性筋膜炎进展凶险且病死率高，需及时手术清除肛周坏死组织。噬血细胞综合征发病常与感染相关。本文报道1例肛周坏死性筋膜炎并噬血细胞综合征，其病例病情复杂，病因不明，旨在提醒肛肠外科医师遇到肛周坏死性筋膜炎患者在反复高热且白细胞计数持续偏低的情况下，应尽早行骨髓穿刺及基因检测证实，以免延误治疗。

Perianal necrotizing fasciitis has a dangerous progression and a high mortality rate, which requires timely surgical removal of perianal necrotic tissue. Hemophagocytic syndrome is often associated with infection. This paper reports a case of perianal necrotizing fasciitis complicated with hemophagocytic syndrome. The case is complicated, and the cause is unknown. The purpose is to remind the anorectal surgeon that in the case of recurrent high fever and persistent low white blood cell count, bone marrow puncture and gene detection should be performed as soon as possible to confirm the diagnosis to avoid delaying treatment.