We cared for a patient with neonatal lupus erythematosus with cutaneous and hepatic involvement that was clinically and histologically indistinguishable from erythema gyratum atrophicans transiens neonatale, an entity described by Gianotti and Ermacora in 1975, which we consider to be a subtype of cutaneous neonatal lupus erythematosus. The differential diagnosis of any annular erythema in the newborn requires careful clinicopathologic evaluation, with direct immunofluorescence studies and determination of anti-Ro/SS-A, anti-La/SS-B, and anti-nRNP antibodies in both the baby and the mother.