Abstract Sialoblastoma is a rare congenital epithelial tumor of the salivary gland that is diagnosed at birth or shortly thereafter with significant variability in histologic range and clinical course; hence, for an individual case, it may be difficult to predict the most appropriate therapy [ Cancer 1972;30:459-69; Pediatr Pathol 1988;8:447-52; Br J Plast Surg 2000;53(8):697-699]. We report the case of a 4-year-old girl who had a widely spreading sialoblastoma of the left cheek. We were obligated to widely resect the tumor including the trunk of the facial nerve, superior part of the left maxilla, and the zygoma. Although the patient was operated 3 times in 4 years, invasion of the tumor could not be stopped. The patient died because of respiratory insufficiency and deterioration of her general health.