Covering: this review covers the literature from 1992 to 2018To date, approximately 80 naturally-occurring secondary metabolites which are structurally-related to fumiquinazolines have been isolated, mainly from marine sources. These alkaloids can be classified into twelve different groups and exhibit different structure motifs depending on the amino acids from which they are derived. This review is focused on isolation, structure elucidation, biological activities, biosynthetic pathways, and synthetic studies of these natural products.