Systemic lupus erythematosus (SLE) is not uncommon in Thai children and adults but neonatal lupus erythematosus has never been reported in Thailand. A case of NLE born of a mother without prior history of SLE was reported. He was initially misdiagnosed as congenital self-healing histiocytosis because of seborrheic dermatitis-like lesions, petechiae, hepatosplenomegaly and thrombocytopenia. Features supporting the diagnosis of NLE in this case were presented.