Purpose: Macular pigment (MP) deficit has been described in macular teleangiectasia type 2 (MTA; acquired MP loss) and in Sjogren-Larsson (SLS; hereditary MP deficiency). Central blue light-induced fundus autofluorescence (FAF) and blue light fundus reflectance (BLR) are reflect MP distribution. This study was performed to describe the morphology in SLS and MTA by multimodal imaging to further investigate of FAF and BLR changes in these disorders. Methods: This was a single- cross-sectional, retrospective, observational study on SLS and MTA treated at our institution. In a multimodal retinal imaging dataset, BLR and FAF changes were compared with the optical coherence tomography clinical appearance of the patients' retinas. Results: Multimodal image seven eyes of four patients with SLS and of 25 eyes of 15 patients with included in this study. In MTA, areas of focal FAF increase were mainly associated with retinal pseudocysts and photoreceptor loss and were co- with regions of increased BLR. In SLS, areas of focally decreased FAF with the typical intraretinal glistening dots. Frequently, a spot of increased FAF was visible at the fovea of SLS patients, often presence of pseudocysts or photoreceptor loss on OCT. Conclusion: In MTA different patterns of FAF alterations could be observed. The areas of BLR, which are thought to correlate with MP loss, appeared to have only restricted correlation with FAF appearance.