Refractory epilepsy is a rare, long-term complication in children with acute leukemia who are receiving chemotherapy. A few studies have reported cases of several patients who developed recurrent complex partial seizures after the initiation of chemotherapy. In these cases, the cause of the refractory seizures was identified as mesial temporal lobe sclerosis. Here, we report on two patients with extratemporal lesions accompanied by refractory seizures, a long-term complication of acute lymphocytic leukemia. Using presurgical evaluations and measures of the surgical outcomes, the lesions were identified as epileptogenic and were located in the mesial temporal lobe. The underlying pathophysiological background is discussed to aid in understanding this uncommon long-term complication. © 2013 The Authors.