A 16-year-old boy recently diagnosed with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) developed an acute infarct in the left pontine region. No relevant abnormalities were found in the brain and cervical angiography, echocardiography, cerebrospinal fluid, and blood samples. Funduscopically, lesions were multiple circumscribed, creamy yellow patches, flat lesions at the level of the retinal pigment epithelium, and the fluorescence angiography confirmed an APMPPE. All visual and neurological symptoms reverted completely after use of steroid. Our patient is the youngest patient with APMPPE and stroke described so far, being the common age presentation between 20 to 40 years.