**Abstract**

Graves'-Basedow's disease (GD) is a well-defined hyperthyroid disorder caused by circulating antibodies that results the overproduction of thyroid hormones. All but a few children present with some degree of thyroid gland enlargement and most have two or more signs of excessive thyroid activity, such as tremor, irritability/ nervousness, tachycardia etc. Fully developed clinical picture is easy to recognize while often the onset is insidious. Thyroid hormones affect many body systems, so signs and symptoms of Graves' Disease can be wide ranging. A survey on PubMed literature was conducted to gather all published pediatric Graves-Basedow's cases with unusual presentation at the time of diagnosis. We found all together 70 manuscripts with relevant information from 1978 to 2020 but mainly adult cases. One third of them were found to meet the criteria we focused on and were included in this paper, though in some situation the unusual findings do not consist part of hyperthyroidism, the rare manifestation is only a coexistence, or the serious disease even precedes the GD. Dermatopathy, hepatic dysfunction, impaired fluid balance, concomitant disorders in thyrotoxicosis, tricky laboratory findings, a phenomenon of metamorphic thyroid autoimmunity, peculiarities of thyroid dysfunction in children with Down syndrome, apparent associations, and reconstitution GD are highlighted in this chapter. Awareness about the relation of these remote findings to GD, or frequent coexistence with GD is important for early diagnosis. Finally, a reasonable suspicion for Graves' Disease may ultimately help to prevent unnecessary investigations and treatment.

**Keywords:** hyperthyroidism, thyrotoxicosis, thyroid autoimmunity, antibodies, rare disorder, Hashimoto thyroiditis, Turner syndrome, Down syndrome, reconstitution GD, children
