Angiokeratoma is a superficial dilation of a blood vessel. A solitary manifestation like this is often removed as a suspected melanoma. Multiple angiokeratomas may occur, e.g., in the genital region of elderly people, but an extensive spread in the area of the buttocks and lower abdomen in a relatively young patient may be the crucial hint for the diagnosis of the rare Fabry’s disease. This X-chromosome linked hereditary lipid storage disease leads to a multifaceted clinical picture and organ damages that significantly shorten the life expectancy. Early diagnosis is necessary because enzyme replacement therapy can slow down the progress of the disease.