Background and Aims: Steven-Johnson Syndrome (SJS) is a rare and severe form of erythema exudative multiforme. Multisystem involvement in SJS and the suspicion of precipitation of the disease with exposure to anesthetic drugs makes anesthesia a challenging task. The concerns during anesthesia are the mucosal lesions and special care that is required to prevent injury to the oropharynx and larynx during airway management and also the drugs used for anesthesia. In the literature, very few isolated case reports or case series are available. Here, we have analyzed the cases of SJS coming for ophthalmic anesthesia, taking into consideration factors like mode of presentation, precipitating factors, associated diseases, types of anesthesia, anesthetic modifications, and various drugs used during anesthesia. Material and Methods: The electronic medical records of 497 cases of SJS who required interventions like ophthalmic examination or surgery (either under local or general anesthesia) over a period of 18 months were analyzed retrospectively. The records were reviewed to obtain the concerned details like anesthesia-inducing agents, muscle relaxants, inhalational agents, and analgesics. The problems concerned with monitoring and intubation were also noted. The data were analyzed and presented as frequency and percentage. Results: Patient age ranged between 9 months and 72 years. Many surgeries were conducted under general anesthesia (441) although a few required local (peribulbar block) anesthesia (56). The drugs administered for general anesthesia were sevoflurane, isoflurane, propofol, thiopentone, vecuronium, and atracurium and those administered for pain management were fentanyl, tramadol, butorphanol, and paracetamol. The patients who were sensitive to nonsteroidal anti-inflammatory drugs (NSAIDs) and paracetamol were not administered the same. None of the patients reacted adversely to the different drugs used for anesthesia. Conclusion: Identifying the precipitating factors, understanding the pathophysiology and its implications for anesthesia will help in successfully managing anesthesia in the rare cases of SJS.