Background: Phakomatosis pigmentovascularis(PPV) is a rare congenital cutaneous condition. It is associated with widespread capillary malformation and pigmented nevus. Objectives: This study aims to evaluate the characteristics and treatment of PPV. Methods: We retrospectively reviewed PPV patients visited our special clinic between 2003 and 2017. Treatment outcome of cutaneous lesions was scored using a 4-point scale. Results: 52 patients (19M/33F) were involved in this study. Type IIa was the most common type and 17.3% patients have systemic involvement. Significantly more systemic involvement was observed in patients with nevus of Ota and in patients with pigmented nevus on head and neck (P = 0.004 and P = 0.035, respectively). 28.6% of the patients’ capillary malformation was almost cleared by pulsed dye laser, whereas 23.7% of the patients’ pigmented nevus was almost cleared and 42.1% of the patients’ pigmented nevus was completely cleared by Q-switched Nd:yttrium-aluminum-garnet laser. The treatment outcome shows significant negative correlation with the extent of port-wine stain or pigmented nevus (P=0.047 and P=0.011, respectively). Conclusion: In PPV patients, pigmented nevus rather than capillary malformation showed better treatment response to lasers. In both capillary malformation and pigmented nevus, smaller lesions likely to have better treatment outcomes.