Solitary angiokeratoma has rarely been described in oral mucosa, mainly in the tongue, where the main concern is either aesthetical or due to bleeding problems. We present a case of solitary angiokeratoma of the tonsillar pillar in a 68-year-old man. Histologically, the morphology was typical of angiokeratoma. It showed an immunohistochemical pattern in consonance with a blood vessel origin, with expression of CD31, CD34, and von Willebrand factor. The lesion did not express D2-40. No other malformation or metabolic disorder was found in the patient. The lesion was surgically removed and due to the disproportionate post-surgery bleeding, the patient was studied by the Hematology Service, and she was diagnosed as an inhibitor of Factor VIII carrier.