OBJECTIVE To report an uncommon case of a 29-year-old Caucasian male diagnosed with unilateral choroidal neovascularization (CNV) secondary to punctate inner choroidopathy leading to visual impairment. METHODS This is a retrospective and descriptive case report based on data from clinical records, patient observation and analysis of ancillary diagnostic tests. RESULTS A 29-year-old Caucasian male presented to our emergency department with complaints of decreased central vision in his left eye (LE), detected a few hours before. Best-corrected visual acuity (BCVA) in the LE was counting fingers at 50 cm and BCVA in the right eye was 20/20. Fundoscopy of the LE evidenced multiple round and yellowish lesions in the macula and nasal to the optic nerve, without intraocular inflammation signs. Optical coherence tomography showed increased retinal thickness with detachment of the neuroepithelium and a slight retinal pigment epithelium detachment. Fluorescein angiography revealed hyperfluorescent lesions with blurred borders in the macula. An intravitreal injection of bevacizumab (1.25 mg/0.05 ml) was administered in the LE, which resulted in anatomic and visual improvement. CONCLUSIONS We present a rare case of unilateral CNV secondary to punctate inner choroidopathy in a young, myopic male.