Keratoconus (KC) shows several distinctive features in clinical appearance, disease progression, and treatment in children compared with adults. Therefore, diagnostic, clinical care, and therapeutic approaches are different. However, pediatric keratoconus is often undiagnosed and thus untreated in many cases. Once diagnosis has been made, compliance with treatment recommendations is often poor. Pediatric keratoconus also tends to have more rapid progression than in adults; therefore, early detection and treatment are paramount to prevent serious vision impairment, which can affect the child's development. This review of pediatric keratoconus discusses important issues such as worldwide epidemiology, clinical features in children compared to adults, and challenges in diagnosis and treatment and focuses on the most appropriate management strategies based on the best available current evidence.