Photoluminescence (PL) upconversion is a phenomenon involving light-matter interactions, where the energy of emitted photons is higher than that of the incident photons. PL upconversion is an intriguing process in two-dimensional materials and specifically designed 2D heterostructures, which have potential upconversion applications in optoelectronic devices, bioimaging, and semiconductor cooling. In this review, we focus on the recent advances in photoluminescence upconversion in two-dimensional materials and their heterostructures. We discuss the upconversion mechanisms, applications, and future outlook of upconversion in two-dimensional materials.