Optical coherence tomography (OCT) is an essential diagnostic modality for macular diseases. However, conventional OCT is limited by ocular aberrances, with a lateral resolution of only 15 to 20 μm, which prevents cellular-level imaging. Adaptive optics optical coherence tomography (AO-OCT) achieves real-time aberration correction, yielding an isotropic three-dimensional resolution of 2 to 3 μm and enabling in vivo visualization of photoreceptors, retinal pigment epithelial cells, and retinal microvessels. In this paper, the technical principles, developmental history, and clinical application value of AO-OCT in macular diseases including age-related macular degeneration, hereditary macular dystrophies, and diabetic macular edema were systematically reviewed. Meanwhile, the advantages and disadvantages of AO-OCT are compared with those of conventional OCT, optical coherence tomography angiography (OCTA), and adaptive optics scanning laser ophthalmoscopy (AO-SLO). In the future, AO-OCT is expected to become a pivotal tool for early diagnosis, precise subtyping, longitudinal monitoring, and therapeutic efficacy evaluation of macular diseases, demonstrating promising prospects for clinical translation.