Abstract: Tang Jinling, Shu Ye, Chen Weijian, Luo Yongqi, Tang Jianping. Department of Dermatology, Hunan Children′s Hospital, Changsha 410007, China Corresponding author: Tang Jianping, Email: jpingtang@hotmail.com 【Abstract】 A girl who aged eight years and seven months presented with prunosus patches on the right buttock for 8 years, gradual unilateral enlargement of the right lower limb for more than 7 years, and multiple vegetations for 1 year． Dermatological examination showed nevus flammeus and multiple malodorous vegetations over the right lower limb with high skin temperature. The right lower limb was thicker and longer than the left lower limb. X-ray examination, magnetic resonance imaging and Doppler ultrasound examination revealed high-flow vascular malformations. Pathological examination of the vegetations showed vascular proliferation, fibroblast proliferation and erythrocyte extravasation. She was diagnosed as Parkes-Weber syndrome accompanied by pseudo-Kaposi′s sarcoma.

Key words: Parkes-Weber syndrome, Pseudo-Kaposi′s sarcoma, Arteriovenous fistula