Sialoblastoma is defined as a rare, congenital or perinatal, aggressive and potentially low-grade malignant, basaloid gland neoplasm that occurs in the major salivary glands. We report a case of a congenital sialoblastoma in the left parotid gland, associated with a hepatoblastoma in a female infant. At birth, a huge mass in the left neck and hepatomegaly were noted. Grossly, the neck mass was well-circumscribed, lobulated and gray tan. Microscopically, the tumor was composed of basaloid aggregates of primitive uniform cells with focal ductal differentiation. The liver showed a well-circumscribed gray tan tumor with extensive hemorrhage and cystic change. Microscopically, the liver revealed characteristic findings of hepatoblastoma. To the best of our knowledge, this is the first case of coexistence of a congenital sialoblastoma and a hepatoblastoma, reported in the literature.