Conclusion: In conclusion we report a case of a solitary angiokeratoma of the oral mucosa. Although rare, it should be included in the differential diagnosis when evaluating any lesion in this location and further investigations should be performed to rule out a metabolic or systemic disease. Results: Histopathologically in macroscopy, the cystic lesion was determined as a small part of tissue in white -grey color, in sizes of 0,6×0,5×0,3 cm. In microscopic evaluation, the epithelial tumor cells were formed mostly tubular, partially in solid form and broadly making pseudopapillary pattern in stromal tissue. In the luminal structures formed by tumor cells, there has been mucin as dark basophilic stained with PAS/Alcian blue (PH: 2, 5). The tumor cells are strong and have common positivity with CK7, and have negativities with CK20 (B-SA method). In the focal area that the tumor cells got the solid pattern, synaptophysin and chromogranin to evaluate the neuroendocrine differentiation wasn't determined.