Sturge-Weber syndrome belongs to the group of neuroektomesodermal diseases, so called facomatoses. In contrast to other diseases of this group, there are not any proofs of the genetic determinated background of the syndrome, as well as the higher frequency of cancer. However, the higher frequency of benign astrocytomas was observed. The presented case of the coincidence of the Sturge-Weber syndrome and acute lymphoblastic leukemia is the second case described in the literature. The authors present the therapeutical problems, associated with glaucoma and epilepsy in the course of this syndrome.