HDL-C is synthesized in the liver and small intestine and is mainly formed by apolipoprotein A-I (ApoA-I) and apolipoprotein A-II (ApoA-II). The newly secreted ApoA-I must acquire cholesterol or phospholipids in order to form pre-βHDL. This step is mediated by ATP binding cassette transporter A1 (ABCA1). Tangier Disease results from mutations in ABCA1 gene that codes for ABCA1 transporter and is characterized by severe deficiency or absence of HDL-C in the circulation that results in accumulation of cholesteryl esters throughout the body, particularly in the reticuloendothelial system. Tangier Disease was also shown to cause cause central nervous system ischemia, stroke and sometimes bleeding. Even heterozygotes for ABCA1 mutations were shown to have increased carotid intima–media thickness. Also platelet abnormalities, including thrombocytopenia, a mild bleeding tendency, altered platelet morphology, and impaired platelet function, which have been reported in Tangier disease might contribute to bleeding in CNS. We present a 17-year-old female patient with stroke due to Tangier disease