Rowell syndrome (RS) is a disorder characterized by the occurrence of erythema multiforme-like lesions (EM) coexisting with lupus erythematosus. It may be considered a rare subtype of lupus-specific skin lesions. Here we report a clinical presentation of RS in a 34-year-old woman with diagnosed systemic lupus erythematosus (SLE), and without any cutaneous lesion previously that presented with skin lesions and fever. Clinical, laboratory, and histopathology assessments confirmed the diagnosis of RS in the context of SLE flare-up. She was treated with methylprednisolone 1g IV daily for 3 days, then continued with oral Prednisolone, Hydroxychloroquine, and Mycophenolate mofetil. Over treatment Skin’s lesions and oral ulcers subsided gradually and disappear all skin lesions after a month, without a scar.