We report a 52-year-old patient with cutaneous angiomas on the trunk in association with angiotropic lymphoma involving the nervous system. The lesional skin showed proliferation of atypical B cells in the capillaries. The diagnosis of angiotropic lymphoma with neurological deficits is often difficult before death. Thus, skin biopsy in this case proved to be diagnostic of the central nervous system disease without the risk of brain biopsy. The diagnosis of angiotropic lymphoma in the future may be achievable by biopsy of coincidental angiomas.