Platelet-rich plasma (PRP) therapy has been widely used for the recovery of musculoskeletal lesions and skin rejuvenation. The clinical use of PRP is based on the increase in the concentration of growth factors and in the secretion of proteins which maximize the healing process at the cellular level. Herein, we report a case of phakomatosis pigmentovascularis (PPV) with chronic stasis ulcer treated successfully with PRP therapy. A 34-year-old male patient with known PPV, characterized by the coexistence of pigmentary diseases (nevus of Ota and Mongolian spots) and nevus flammeus, came to our dermatological department with asymptomatic ulcer on his right lower leg for 2 weeks. He had a long history of recurrent ulcer on the right lower leg due to venous insufficiency as a systemic involvement of PPV and had been treated with split-thickness skin graft. As the lesion was refractory to 2 months of conservative treatment such as wearing elastic stocking, debridement and dressing, we applied PRP therapy every 1~2 weeks for 2 months and the lesion improved remarkably.