Herpes zoster ophthalmicus (HZO) is a rare presentation of herpes zoster in children; however, it may become chronic and debilitating. The pathophysiology of HZO complications is not completely understood and may include virus virulence, vascular and neural inflammation and immune reactivity. Therefore, clinical experts suggest an antiviral agent combined with topical steroids, but treatment duration and the need for secondary prophylaxis, given the likelihood of recurrence, are not clearly defined. We present a complex case of HZO in a varicella zoster virus (VZV)-vaccinated toddler successfully treated with acyclovir and topical steroids. We also present a review of the relevant literature regarding the therapeutic management and long-term sequelae of HZO in children.