PURPOSE: To report the diagnosis, management, and outcome of acute retinal necrosis syndrome in children. METHOD: Case series of three consecutive children aged 11 years and younger who were diagnosed with acute retinal necrosis. In addition to full ocular and systemic examinations, the children underwent vitreous biopsy (Patients 1 and 2) or aqueous tap (Patient 3) for polymerase chain reaction analysis. RESULTS: All patients had unilateral retinitis that was associated with preexisting chorioretinal scars, and two patients (Patients 1 and 3) had concurrent extraocular central nervous system abnormalities. Intraocular herpes simplex virus was detected in all three children: Type 1 in Patient 1 and Type 2 in Patients 2 and 3. In addition, all three children had a history of extraocular herpes simplex virus infection. CONCLUSIONS: Retinitis associated with preexisting chorioretinal scars and detectable intraocular herpes simplex virus on polymerase chain reaction was common to all three children with acute retinal necrosis.