Sialolipoma is a rare tumor that occurs in the head and neck. We present a case arising from a minor salivary gland in an infant. The 6-month-old infant presented with difficulty swallowing, frequent reflux, and snoring that had been worsening several weeks before presentation. Physical examination showed a large mass arising from the tongue base. The patient was taken to the operating room for transoral removal of a presumed cyst. Histologic examination of the lesion showed a well-circumscribed lesion composed of lobules of mature adipose tissue and nodules of entrapped, non-neoplastic acini and ductules separated by thin, fibrous septae, consistent with a sialolipoma. To the best of our knowledge, this is the first reported case of a sialolipoma at the tongue base in a child. The patient has had no evidence of recurrence at 1 year of follow-up.