BACKGROUND: Toxic epidermal necrolysis (TEN) is a rare life threatening dermatological disorder characterized by extensive epidermal detachment and erosion of mucous membranes. It is typically a side effect of some medications. Nevirapine, a nonnucleoside reverse transcriptase inhibitor (NNRTI) is one of the frequently used components of highly active antiretroviral therapy (HAART). Skin rash is its common adverse reaction, usually mild and rarely progressing to TEN. Ophthalmic involvement is common as well but rarely progresses to blindness especially in the pediatric population. CASE PRESENTATION: We report the case of a 3 year 5 month old child diagnosed with HIV who developed TEN 8 days after starting a Nevirapine based HAART regimen. Drug withdrawal and supportive treatment alone were the modalities employed to achieve complete re-epithelization of lesions. Patient was lost to follow-up 6 months after being in care and was only seen 3 years later with total loss of vision. CONCLUSION: Blindness, though rare, can be a long-term complication of TEN in children especially with HIV infection. Physicians and patient caregivers should closely monitor these patients, especially during their early stages of treatment amongst others for development of adverse drug reactions. Long-term retention in care is pivotal for identification and prompt management of ocular and other chronic complications, albeit recognizing management challenges in low resourced settings.