Central hypothyroidism is a rare condition characterized by insufficient production of thyroid hormones by the thyroid gland caused by defects in TSH secretion from the pituitary or TRH secretion from the hypothalamus. Its etiology includes genetic and acquired causes, mainly associated with tumors in the hypothalamic-pituitary region or treatments such as surgery and radiotherapy. This review discusses advances in its etiology, diagnosis, and treatment, with a focus on diagnostic and therapeutic challenges. It highlights the importance of measuring concomitant free T4 and TSH for early detection and preventing neurological sequelae, particularly in congenital central hypothyroidism. Associated risks include growth and developmental impairment, as well as metabolic and cardiovascular alterations. Levothyroxine treatment is essential for correcting hormonal imbalances and improving quality of life, though caution is needed to avoid overdosing, especially in vulnerable populations. The importance of personalized treatment and continuous monitoring is emphasized to ensure the best clinical outcomes and promote optimal quality of life.