Optic disc drusen (ODD) are benign calcified deposits, which are located at the head of the optic disc. Most ODD patients are asymptomatic. Ocular complications, related to ODD, are considered rare. Optic disc drusen, especially if it is bilateral, may mimic the clinical presentation of papilledema. The clinical discrimination between ODD and papilledema may be a challenging task, and in many cases, especially in the pediatric population, an ocular ultrasound examination is needed. A fundus examination, in order to rule out papilledema, is a common daily task for the ophthalmologist and is considered perhaps the most common interface between pediatricians, neurologists, internal physicians and ophthalmologists. In this review, we focused on the pathogenesis, epidemiology, diagnosis and clinical implications of ODD.