Background/Objectives: Macular Telangiectasia Type 2 (MacTel type 2) is a rare, progressive retinal disease that can lead to central vision loss. Optical coherence tomography (OCT) plays a crucial role in the early diagnosis, monitoring, and prognostic assessment of this condition. This narrative review aims to summarize the clinical features, OCT findings, and current management strategies for MacTel type 2. Methods: A literature search of PubMed, MEDLINE, and Google Scholar was performed for articles published from October 1993 to September 2025 using keywords related to MacTel type 2, OCT, clinical features, and treatment. All relevant clinical studies, including observational studies, clinical trials, and case series, were considered. The literature was screened independently by two authors, and a total of 69 articles were included. Results: Characteristic OCT findings include foveal cavitation, hyperreflective middle retinal layers, inner and outer retinal cavities, ellipsoid zone disruption, and retinal pigment clumps. Central macular thickness is consistently reduced, and structural biomarkers identified on OCT correlate with visual acuity decline. Treatment strategies vary by disease stage: non-proliferative MacTel type 2 currently has no universally effective therapy, although neuroprotective interventions such as ciliary neurotrophic factor (CNTF) show promising results. Proliferative MacTel type 2 is primarily managed with anti-vascular endothelial growth factor therapy (anti-VEGF), demonstrating functional and anatomical improvements. Conclusions: OCT provides essential structural evaluation for monitoring MacTel type 2, while treatment approaches remain stage-dependent. Emerging therapies, including CNTF implants and novel anti-VEGF agents, hold potential for improving outcomes.