In this report, we describe a rare case of a Nigerian male infant who presented with resistance to thyroid hormone associated with hyperthyroidism, cholestatic jaundice, a huge goitre and failure to thrive (weight faltering). He had delayed developmental motor milestones and at the age of 7 months, he has developed craniosynostosis. The hyperthyroidism persisted despite treatment with beta-blocker for tachycardia and antithyroid medications. The challenges encountered in the management of the patient are discussed