We report on a case of mucoepidermoid carcinoma of the parotid gland following treatment of acute lymphoblastic leukemia (ALL) in childhood. The female patient was diagnosed as having T-cell ALL at the age of 13 years. Treatment included multidrug chemotherapy and prophylactic 18 Gy cranial irradiation. She developed low-grade mucoepidermoid carcinoma of the right parotid gland at the age of 24 years, the most probable cause of the secondary malignancy being radiation. In a literature review of mucoepidermoid carcinoma following ALL, 11 out of 14 cases received radiation therapy for the initial treatment of ALL, but 3 cases had no radiation therapy. The parotid gland carcinoma as a secondary malignancy following ALL in childhood is rare, but it highlights the need for concern about the secondary malignancy in patients with painless parotid swelling after chemoradiotherapy.