The case of a 38-year-old white man with a chondroid syringoma in the left side of the upper lip is reported. Histochemical study of the lesion showed that the morphologic distributions of the neutral mucosubstances, sulfated acid mucopolysaccharides, and elastic fibers in the reported case were similar to those of mixed salivary gland tumor. The recently reported ultrastructural and immunohistochemical features of chondroid syringoma are also discussed in an effort to clarify its histogenesis.