Summary Background: Despite long-standing iodine supplementation in Iran, the prevalence of goiter remains high in some areas. This suggests other nutritional deficiencies may be considered as responsible factors for goiter persistence. In the present study we investigated the possible role of vitamin A deficiency (VAD) and low vitamin A status in the etiology of endemic goiter in Semirom, Iran. Materials and Methods: In this cross-sectional study, 1,828 students from 108 primary schools of urban and rural areas of Semirom were selected by multistage random cluster sampling. Thyroid size was estimated in each child by inspection and palpation. Urinary iodine concentration (UIC) and serum retinol (SR) were measured. Results: Overall, 36.7% of schoolchildren had goiter. The median UIC was 18.5 g/dL. The mean Ϯ SD of SR in goitrous and nongoitrous children was 38.84 Ϯ 10.98 and 39.17 Ϯ 10.85 g/dL respectively ( p ϭ 0.82). There were two children with VAD (SR less than 20 g/dL); one in the goitrous and one in the nongoitrous group. The prevalence of subjects with low vitamin A status (SR less than 30 g/dL) in the goitrous and nongoitrous groups was 26.2 and 21.5% respectively ( p ϭ 0.42). Conclusion: Goiter is still a public health problem in this region. Iodine deficiency, VAD or low vitamin A status is not among the contributors of goiter persistence in schoolchildren of Semirom. The role of other micronutrient deficiencies or goitrogens should be investigated. Key Words goiter, iodine deficiency, vitamin A deficiency, low vitamin A status Iodine deficiency (ID) is recognized as the major preventable public health problem worldwide. It is estimated that 750 million people worldwide are at risk of iodine deficiency disorders (IDD) ( 1 ). IDD can be presented with a wide variety of clinical manifestations ranging from congenital anomalies, cretinism, deaf mutism, psychomotor defects and severe hypothyroidism to goiter ( 2 ). Vitamin A deficiency (VAD) is a major public health problem too; those most vulnerable include preschool children and pregnant women in low-income countries. In children, VAD is the leading cause of preventable visual impairment and blindness ( 3 ). In a meta-analysis in 2002, it was estimated that there were 127.2 million vitamin A (VA) deficient preschool-aged children, representing 25% of preschoolaged children in high-risk regions of the developing world ( 4 ). These deficiencies often coexist in children in developing countries ( 5 ).In animals, VAD has multiple effects on thyroid metabolism ( 6 ). In the thyroid, VAD decreases thyroidal iodine uptake and iodine incorporation into thyroglobulin (Tg) and increases thyroid size ( 7 -11 ); in the periphery, VAD increases circulating thyroid hormone concentrations ( 12 ); and in the pituitary, VA status modulates thyrotropin (TSH) production by retinoid X receptor (RXR)-mediated expression of pituitary TSH- mRNA ( 12 -18 ), and VAD in rats increases pituitary TSH- mRNA, TSH, and circulating thyroid hormone ( 12 ). High-dose VA supplementation...