We report a young woman with genetically confirmed Pendred syndrome and discuss 
the current therapeutic strategies of dyshormonogenetic goitre. A small diffuse 
thyroid enlargement developed during infancy and although substitution therapy 
with L-thyroxine was adequate, it progressed and underwent multinodular 
transformation. Cervical ultrasound at the age of 22 years demonstrated three 
solid nodules and fine-needle aspiration biopsy showed a finding typical of 
follicular adenoma. It is known that dyshormonogenetic goitres have a tendency 
to grow despite appropriate treatment with L-thyroxine. Management of a patient 
with Pendred syndrome requires careful follow-up and regular imaging of the 
thyroid. Although the therapeutic approach to dyshormonogenetic goitres is still 
controversial, in our patient we chose total thyroidectomy as the most 
advantageous method to prevent the development of malignancies that may arise 
more frequently from dyshormonogenetic goitres than from goitres of other 
aetiologies.