Congenital toxoplasmosis is a potentially devastating congenital infection caused by Toxoplasma gondii. Pregnant women may acquire primary infection after exposure to cat feces, by ingestion of oocysts on unwashed fruits and vegetables, or by ingestion of bradyzoites in raw or undercooked meat. Infection in pregnant women is usually subclinical or mild; in the absence of screening programs, the diagnosis is rarely made clinically. Fetal infection can result in hydrocephalus, intracerebral calcifications, and chorioretinitis; however, like most congenital infections, the majority of infants with congenital toxoplasmosis are asymptomatic in the newborn period. Treatment of infected infants with combination antiparasitic therapy for 1 year can dramatically improve clinical outcomes. The primary long-term morbidity of congenital toxoplasmosis includes ocular reactivations and central nervous system injury. Prevention of congenital toxoplasmosis is based on education of exposure risks and antiparasitic therapy during pregnancy if infection is identified.