Chondrolipomas are benign mesenchymal tumors characterized by the proliferation of mature adipocytes associated with variable amounts of mature cartilaginous tissue. We describe here a case of chondrolipoma of the tongue in a 30-year-old man. The patient reported the presence of the lesion for approximately 10 years. Intraoral clinical examination revealed a nodular, sessile, pink mass of firm consistency, which was located along the midline of the posterior region on the dorsal surface of the tongue. An excisional biopsy was performed and histopathological examination revealed a well-delimited proliferation of mature adipocytes arranged in lobules and separated by fibrous connective tissue septa. Islands of mature cartilaginous tissue were identified in close proximity to the fibrous connective tissue septa, as well as lacunae filled with chondrocytes. No myxoid areas, lipoblasts, atypias, hyperchromasia or mitosis were detected. After 5 years of follow-up, the patient shows no signs of recurrence. Chondrolipomas are uncommon in the oral cavity, with only nine cases being reported in the international literature (Pubmed Database). (J Oral Sci 51, 313-316, 2009)