Neonatal lupus erythematosus (NLE) is a disease primarily characterized by cardiac and/or cutaneous involvement. Hepatic, hematological, neurological and pulmonary involvement are rare manifestations and normally considered as mild and transient complications. But recent studies have shown more frequent hepatic involvement in NLE. We report a two month-old male infant, born to a clinically asymptomatic mother, presenting with significant hepatic involvement and annular, erythematous plaques with hyperkeratotic borders at the eyebrow region and anterior surface of trunk. Both the infant and his mother were positive for anti-Ro (SS-A) and anti-La (SS-B).