Premature ovarian insufficiency (POI) is characterized by the cessation of ovarian function before the age of 40. Etiology is multifactorial, including genetic, autoimmune, iatrogenic, metabolic, infectious, and environmental factors. Seventy-five to ninety percent of cases may be idiopathic. The diagnosis is based on elevated serum FSH (>25 IU/L) on two occasions, at least 1 month apart. POI manifests mostly as secondary amenorrhea or as primary amenorrhea. Estrogen deficiency causes vasomotor, genitourinary, and neuropsychiatric symptoms and long-term systemic complications like osteoporosis and increased risk of fractures, cardiovascular diseases, and metabolic dysfunction. Infertility is the other distressing symptom. The management aims to reduce estrogen deficiency symptoms, prevent long-term complications, and address fertility concerns. Hormone replacement therapy (HRT) with estrogen-progesterone until the natural age of menopause (~50 years) is the mainstay of management. Oral contraceptive pills can be used for hormone replacement; however, its benefit over sequential HRT for bone health is questionable. Annual assessment of blood pressure, lipid profile, and metabolic parameters is recommended. Dual-Energy X Ray Absorptiometry (DXA) helps in baseline bone marrow density (BMD) assessment and is repeated not before 2 years. Spontaneous pregnancy is rare and mostly occurs in the early years of diagnosis. Oocyte donation with in vitro fertilization (IVF) is the most effective fertility treatment.