A 19-year-old woman with a medical history of depressive mood arrived and was treated with lamotrigine at 25 mg/day. On day 10, a high fever of 39.3 °C and a diffuse, erythematous, pruritic full-body rash involving the palms of her hands and the soles of her feet developed, and she was diagnosed with Stevens-Johnson syndrome (SJS). On day 17, white blood cell count (WBC) result was 1,240/μl with 54.1% neutrophils (670/μl), and the WBC decreased to 840/μl with 60.7% neutrophils (510/μl) on day 18. The trend toward improvement included skin symptoms after steroid pulse therapy using 1000 mg/day. Based on the clinical course, we concluded that the SJS and leukopenia and/or neutropenia are associated with lamotrigine. Monitoring of WBC should be kept in mind when administering lamotrigine.