Background: Galactosyltransferases (Gal-T) are intracellular and cell surface enzymes involved in the biosynthesis of glycoconjugates. Gal-T were suggested to be implicated in oncogenesis, since their levels are elevated in various body fluids of patients with different types of malignoma; the invasiveness of certain neoplastic cell lines is correlated with their cell surface Gal-T activities, and cancer-associated Gal-T-isoforms were identified. Material and Methods: Gal-T activities in the cerebrospinal fluid (CSF) of patients with meningeal involvement in acute leukaemia or high-grade lymphoma (n = 8), normal controls (n = 54), patients with acute viral meningitis/encephalitis (n = 8) and multiple sclerosis (n = 10) were compared. CSF Gal-T activities were measured with uridine diphosphate-14C-galactose as substrate after isolation of the enzymatically transferred 14C-galactose by high-voltage electrophoresis. Simultaneously, the permeability of the blood-CSF barrier was analysed by the Q-albumin method. Results: In case of an intact blood-CSF barrier, Gal-T activities were elevated exclusively in patients with meningeal involvement in malignancies, but declined rapidly during intrathecal polychemotherapy. Conclusions: CSF Gal-T activities might prove to be a marker for early or residual involvement of the central nervous system in acute leukaemia or high-grade lymphoma.