Placoid pigment epitheliopathy and serpiginous choroiditis are among the white dot retinal syndromes and possess similarities that can cause confusion between these two diseases. However, they are very different in terms of their progression and prognosis, which requires a diagnosis of certainty in order to better manage the patients with the diseases and identify potentially serious progressive complications. The clinical presentation, results of testing, differential diagnoses and treatment of these two pathologies are discussed in this article.