Optic nerve head drusen are acellular, calcified deposits which can be found in buried drusen form or exposed drusen form. Choroidal neovascularization caused by optic nerve head drusen is very rare in children. This case reports a 13-year-old girl with unilateral choroidal neovascularization caused by optic nerve head drusen. She was referred to us with decreased visual acuity in her right eye occurring 6 weeks previously. The best-corrected visual acuity in the right eye was 20/80. Dilated fundus examination revealed elevated optic discs with blurred margins in both eyes. In addition, an elevated yellow lesion extending from the optic nerve head toward the macula was observed in the righ eye. Fundus autofluorescence imaging demonstrated bright nodular autofluorescence corresponding to optic nerve head drusen on the surface of optic nerve head in both eyes. In the right eye, a central area of relative hypoautofluorescence surrounded by marked hypoautofluorescence from choroidal neovascular membrane and/or subretinal fluid/fibrinous exudate located temporal side of the optic nerve head. The late phase of a fluorescein angiography scan showed a central area of hyperfluorescence corresponding to choroidal neovascular membrane surrounded by blocked fluorescence from subretinal fluid/fibrinous exudate in the right eye despite normal findings in the left eye. Spectral domain optical coherence tomography imaging showed irregular bulges over the area of optic nerve head in both eyes. The patient was diagnosed as juxtapaillary choroidal neovascular membrane secondary to optic nerve head drusen. 0.5 mg/0.05 ml intravitreal ranibizumab injection was then performed under general anesthesia. After a month from the injection, BCVA increased to 20/25. Serial scans of optical coherence tomography at month 1, month 3 and month 9 showed no subretinal fluid. The best-corrected visual acuity maintained at the same level (20/25) and no complication related to the injection was observed. Optic nerve head drusen may lead to loss of visual acuity in children. Choroidal neovascularization caused by optic nerve head drusen may be treated by intravitreal ranibizumab injection. Pro re nata regimen should be advised in pediatric patients.