An 11-year-old Chinese girl had bluish-gray hyperpigmented patches combined with two dark brown papules on her left face since birth, and she developed a hypopigmented patch within the bluish-gray patches after sun exposure at the age of eight. Her previous medical history suggested that the hypopigmented patch was sensitive to the treatment of tacrolimus ointment cream and 308-nm excimer laser, while the hyperpigmented patches were insensitive to the previous initial laser treatment. Based on the clinical manifestations and a positive result of Wood's lamp examination, she was diagnosed with nevus of Ota, congenital halo nevus, and vitiligo. Only three cases of the co-occurrence of nevus of Ota and vitiligo within one area have been reported to date, while this is the first case to present a concurrence of congenital halo nevus and vitiligo within the area of nevus of Ota in the literature.