Two-dimensional (2D) transition metal dichalcogenide (TMDC) monolayers, a class of ultrathin materials with a direct bandgap and high exciton binding energies, provide an ideal platform to study the photoluminescence (PL) of light-emitting devices. Atomically thin TMDCs usually contain various defects, which enrich the lattice structure and give rise to many intriguing properties. As the influences of defects can be either detrimental or beneficial, a comprehensive understanding of the internal mechanisms underlying defect behaviour is required for PL tailoring. Herein, recent advances in the defect influences on PL emission are summarized and discussed. Fundamental mechanisms are the focus of this review, such as radiative/nonradiative recombination kinetics and band structure modification. Both challenges and opportunities are present in the field of defect manipulation, and the exploration of mechanisms is expected to facilitate the applications of 2D TMDCs in the future.