Cutaneous polyarteritis nodosa is a form of polyarteritis nodosa. It is a rare disease in children and is characterized by its benign and chronic course. There is no evidence of hypertension or organ dysfunction. The aim of this study was to present a case of cutaneous polyarteritis nodosa and to review the literature. We describe the case of a 3-year-old male child with multiple, red, painful, edematous nodules of the extremities and trunk. Dermatologic findings were accompanied by fever and arthritis. Laboratory findings showed an acute phase response associated with raised antistreptolysin titer. Diagnosis was based on biopsy of the affected skin lesion in which necrotizing arteritis of the lower dermis with neutrophilic and eosinophilic infiltrates was found. The patient responded well to corticosteroid therapy. The evaluation of children with cutaneous polyarteritis nodosa should include laboratory studies to detect streptococcal infection.