Tangier disease (TD) is a rare inherited disorder of lipoprotein metabolism, characterised by the accumulation of cholesterol esters in various tissues, resulting from a marked deficiency or absence of high-density lipoproteins. There is limited information in the literature regarding the impact of TD on reproduction. We present the case of a 34-year-old female patient who was diagnosed with TD 9 years ago with extremely low concentrations of HDL cholesterol (0.7 mg/dL), LDL cholesterol (5 mg/dL) and apolipoprotein A1 (0.02 mg/dL). Genetic analysis revealed a homozygous c.4218delC (p.Asn1406Lysfs*95) pathogenic variant in the ABCA1 gene causing TD. She presented with a mild clinical course that included gastrointestinal involvement and mild thrombocytopenia. Despite being clinically stable for a long time, the patient, who desired to become pregnant, was diagnosed with premature ovarian insufficiency (POI) according to the hormon profile (FSH: 58.7 mIU/mL, LH:37.1 mIU/mL, estradiol: 34.8 pg/mL, Anti-Mullerian hormone: 0.08 ng/mL) after experiencing amennorrhea during follow-up.