Abstract We report a case of Kikuchi’s disease (KD) in a 19year old Saudi woman who initially presented with fever, myalgia, headaches and cervical and supraclavicular lymphadenopathy at the age of 10years. A lymph node biopsy demonstrated the typical necrotizing lymphadenitis of KD. Her disease was controlled with oral corticosteroids and remained quiescent for 7years without treatment. Recently, her systemic symptoms reappeared and she developed an acneiform eruption consisting of scaly, erythematous macules and indurated papules, with no comedones, over face, neck and mid chest. Skin biopsy was consistent with the diagnosis of KD. The majority of cutaneous lesions of KD show the characteristic histological changes that parallel those of the lymph nodes. Few have non-specific clinical and histological findings as this case, and are a diagnostic challenge. Dermatologists should be aware of this rare entity when evaluating acneiform eruptions with concomitant systemic symptoms. Screening and intermittent observation and evaluation for autoimmune disease, specifically systemic lupus erythematosus (SLE) is recommended in confirmed KD.