In order to investigate the nerve distribution in mixed venous-lymphatic malformations (MVLMs), 57 postoperative patients diagnosed with MVLMs of the tongue were selected. Immunohistochemistry staining for neurofilament (NF) was used to detect sensory nerve fibers. Distribution of NF in samples from MVLMs was compared with distribution of NF in normal tongue tissue, venous malformations, lymphatic malformations and venular malformations. Results showed that the number of NF-positive nerve fibers in MVLMs was comparable to that in venous malformations and lymphatic malformations. The number of nerve fibers in MVLMs was significantly lower than in normal tissues. NF distribution in MVLMs was not affected by the patient's age or the coexistence of infection. These data suggest that the decreased distribution of sensory nerve fibers in MVLMs may be involved in the pathogenesis of MVLM of the tongue.