Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life-threatening conditions with a high mortality rate. SJS and TEN denote a group of disorders closely related to each other, characterized by extensive epidermal necrolysis and usually induced by drugs. Keratinocyte apoptosis is the main reason for widespread epidermal detachment. Its etiology usually depends on drugs. Drugs or their metabolites can act as a hapten after binding to the keratinocyte surface and initiate a cytotoxic immunological attack. In this article, a 16-year-old male patient diagnosed with SJS and turned into SJS/TEN overlap with the characteristics of skin lesions during the follow-up, whose complaints improved with treatment and later developed drug-related fever, is presented. Early diagnosis and withdrawal of suspected drugs are some of the most important steps in the treatment. SJS/TEN should be excluded from other diseases similar to SJS/TEN. Although various topical and systemic treatments have recently been used, supportive care is still the most important and effective therapeutic approach. This paper aimed to review clinical findings, with advances in etiopathogenesis and treatment of SJS/TEN and drug fever in light of current literature.