Background: Palisaded neutrophilic granulomatous dermatitis is a rare cutaneous manifestation in children associated with autoimmune pathologies. The exact pathogenesis of this disease is still unknown. However, it has been suggested that the deposition of immune complexes could initiate this pathology. Case report: We describe the case of an 11-year-old female patient who presented with polymorphic lesions in extremities associated with polyarthralgia. She was diagnosed with systemic lupus erythematosus and lupus nephritis. Because of the skin lesions, histopathological and immu-nohistochemical skin studies (CD68, CD163, myeloperoxidase) were performed, which resulted in palisaded neutrophilic granulomatous dermatitis. Due to renal involvement, treatment was administered with methylprednisolone pulses, hydroxy-chloroquine, mycophenolate mofetil, antihypertensives, and nonsteroidal anti-inflammatory drugs. The clinical response was favorable during follow-up. Conclusions: Palisaded neutrophilic granulomatous dermatitis associated with systemic lupus erythematosus is unusual. Therefore, its recognition is important, as it may appear as the initial manifestation of this autoimmune disease.