We describe a term infant with congenital cutaneous candidiasis (CCC), and review all cases in the English literature that reported birth weight and outcome. Presence of an intrauterine foreign body was a predisposing factor for development of CCC and subsequent preterm birth. The most common presentation of CCC in neonates weighing >1000 g was a generalized eruption of erythematous macules, papules, and/or pustules that sometimes evolved to include vesicles and bullae. Extremely low birth weight, premature neonates weighing <1000 g most often presented with a widespread desquamating and/or erosive dermatitis (10 of 15 [67%]), and were at greater risk for systemic infection with Candida spp (10 of 15 [67%]) and death (6 of 15 [40%] than those weighing >1000 g (5 of 48 [10%]; 4 of 48 [8%], respectively). Systemic antifungal therapy is recommended for neonates with burn-like dermatitis attributable to Candida spp, or positive blood, urine, and/or cerebrospinal fluid cultures. Systemic treatment also should be considered for all infants with CCC who have respiratory distress in the immediate neonatal period and/or laboratory signs of sepsis such as an elevated leukocyte count with an increase in immature forms or persistent hyperglycemia and glycosuria.