Several case series have suggested that rituximab is efficacious in adult patients with minimal change disease. We herein report a case of disseminated intravascular coagulation-like reaction after rituximab infusion in a patient with nephrotic syndrome. A 58-year-old Japanese man with minimal change disease diagnosed 15 years earlier was started on rituximab to prevent relapse of nephrotic syndrome when he presented to our clinic with low albuminemia, massive proteinuria, and leg edema. Eleven days after rituximab infusion, he presented with abdominal pain, appetite loss, and tarry stool. A laboratory examination revealed severe thrombocytopenia and coagulopathy, and upper gastrointestinal endoscopy revealed multiple hemorrhagic ulcers in his esophagus and stomach. The patient died two days later. Physicians should consider disseminated intravascular coagulation-like reaction when encountering cases with thrombocytopenia after rituximab infusion for any disease.