Thyroid agenesis and hemiagenesis are rare and continue to be material for publication. Hemiagenesis of thyroid often goes undetected or is detected on screening ultrasound of the neck in school children. Goitrogenesis or goiter in the only remaining lobe is rare. Hence this attempt to publish this case of hemiagenesis with goiter in the only remaining lobe. Objective to add to the literature a case of hemiagenesis of thyroid with goiter in the only remaining lobe. Case details: A 12 year old boy presented with painless nodular goiter on clinical right side. He was on anti-thyroid drugs for a very long tie with no clinical symptoms of hypothyroidism or hyperthyroidism. Goiter was taken up for surgery as it was enlarging in size, causing cosmetic and psychological disturbances. Exploration revealed a single right lobe; isthmus and contralateral lobe were absent. Post-operatively the child was put on lifelong thyroid supplementation. Radioisotope scan confirmed the absence of the contralateral lobe. A rare case of thyroid hemiagenesis with goiter in the only remaining lobe is reported.