SUMMARY Background: Angiokeratoma is an asymptomatic, hyperkeratotic, capillary disorder of the skin present as solitary or multiple, keratotic papules or plaques, which may also be related to Fabry disease. Oral involvement may be observed in cases of widespread muco-cutaneous angiokeratomas, whereas solitary buccal angiokeratoma without systemic/cutaneous involvement is extremely rare. Case Report: A 45-year-old woman was referred with a 3-month, painless, bluish lesion, located on left

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... mucosa. The medical record of the patient was free of any systemic disease or medication. After a careful clinical oral, mucosal as well as skin examination, an excisional biopsy was taken. A routine haematoxylin-eosin staining and additional immunohistochemistry were performed. Differential diagnosis included haemangioma, haematoma or lesions of melanocytic origin. Clinical examination showed a solid, lobulated bluish lesion, located on left buccal mucosa without other skin or mucosal involvement. The microscopic findings revealed dilated vascular spaces covered by normal endothelium without atypia, extending into the epithelium, indicating the diagnosis of angiokeratoma. Conclusions: Despite its rare occurrence, solitary angiokeratoma of oral mucosa should be included in the differential diagnosis of black-bluish lesions. Further investigation for other similar lesions throughout skin or mucosa is needed to avoid complications as haemorrhage.