Weaver syndrome is a rare disorder, characterized by accelerated growth, advanced osseous maturation and distinct craniofacial features. Macroglossia and hypothyroidism are seldom mentioned in the literature as clinical findings associated with the syndrome. This paper describes a patient with Weaver syndrome, referred for consultation and treatment of macroglossia, who also suffered from congenital hypothyroidism. This is the first reported case of Weaver syndrome treated with partial glossectomy (tongue reduction). The paper describes the clinical findings of the syndrome, emphasizing the difficulty in identifying it, the indications for partial glossectomy and the authors' recommended operative technique.