Stevens-Johnson syndrome (SJS) is defined as a severe erythema-multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa and hemorrhagic crusting on the lips; it is associated with fever, headache, and arthralgia. The disease is a part of a continuum of immunologically mediated mucocutaneous diseases at various grades of severity. SJS is often induced by drugs, but the pathophysiologic mechanism is completely unknown. Patients and their first degree-relatives may have genetic defects in their metabolic pathways that lead to the accumulation of toxic metabolites. However, viral infections are known triggers of this skin disorder. Varicella zoster virus has been only very rarely reported as an etiological agent in SJS, despite its high incidence as a pathogen in childhood. In this study, we report a case of primary varicella infection associated with SJS because of its rare presentation. To the best of our knowledge, our case is the youngest child in the literature.