Objective: Review of contemporary knowledge about inherited metabolic disorders in pregnant women and fetuses. Design: A review of literature. Setting: Laboratory for inherited metabolic disorders, Departament of Clinical Biochemistry and Childern’s Clinic, University Hospital and Palacky University, Olomouc. Method: Review of literature and publications using medical databases. Conclusion: Metabolic examination of mother or child is recommended if any clinical problems during or immediately after pregnancy occur. Nowadays most of inherited errors of metabolism can be diagnosed prenataly in first or second trimester of pregnancy.

        Key words: inherited disorders of metabolism, prenatal diagnosis, pregnancy, abortions, HELLP syndrome, AFLP syndrome