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Sarcoidosis is a chronic multisystemic disease, which can be rarely associated with autoimmune disorders, such as antiphospholipid syndrome (APS). Although amaurosis fugax is an uncommon complication, its presentation can unmask a carotid artery dissection (CAD) in these diseases. In addition, central serous chorioretinopathy (CSC) has been related to vascular disorders too. We presented a case of a Caucasian middle-aged man, who developed CAD symptoms, such as amaurosis fugax in the right eye (RE) and headache. His medical history included arterial hypertension, hypothyroidism, and Lofgren's syndrome. On examination, retinal pigment epithelium (RPE) atrophy and subretinal fluid (SRF) in the macular area of the RE were observed. These findings were confirmed by optical coherence tomography (OCT), which also revealed an increase in choroidal thickness. However, these differed significantly from the contralateral eye. These clinical symptoms and imaging findings suggested a CSC in the RE, but not all clinical processes were justified. Subsequently, a CT angiography was performed and confirmed a significant occlusion in the right internal carotid artery and progressive sharpening of the lumen with an intimal flap due to a carotid dissection. In addition, the laboratory results were compatible with antiphospholipid syndrome (APS). To the authors' knowledge, the patient returned to the ED due to an anterior uveitis and he is currently asymptomatic with Cemidon and Adalimumab treatment. We described for the first time a case of carotid dissection and central serous chorioretinopathy in the context of two autoimmune-based pathologies, such as sarcoidosis and antiphospholipid syndrome. APS = Antiphospholipid syndrome, BCVA = Best-corrected visual acuity, CAD = Carotid artery dissection, CNV = Choroidal neovascular membrane, CSC = Central serous chorioretinopathy, CT = Computed tomography, ED = Emergency Department, ICAD = Internal carotid artery dissection, LE = Left eye, OCT = Optical coherence tomography, RAPD = Relative afferent pupillary defect, RPE = Retinal pigment epithelium, RE = Right eye, SRF = Subretinal fluid.