Rationale:Congenital scoliosis due to a hemivertebra creates a wedge-shaped deformity, which progresses and causes severe spinal deformities as an individual grows. The treatment of congenital scoliosis focuses on early diagnosis and appropriate surgical management before the development of severe deformity.Patient concerns:We report the case of a 4-year-old male child with a left thoracolumbar scoliosis of 27° (T10-T12) due to a T11 hemivertebra who was treated by posterior fusion and pedicle screw fixation at the age of 4 years. The implant was removed due to pain secondary to implant prominence after 4 years without definitive revision surgery, which led to significant progression of the scoliosis, to 50°. The indication for posterior vertebral column resection (PVCR) is a congenital spinal deformity with a curve magnitude greater than 30° with fast progression. This includes documented progression of the curve by more than 5° in a 6- month period, failure of conservative treatment, or both.Outcomes:The patient underwent PVCR of the T11 hemivertebra. Nine years after the revision surgery with PVCR, the patient showed satisfactory results and his spine was well balanced.Lessons:This case shows that removal of an implant that was not the only cause of curve progression at a young age may lead to progression of scoliosis and, therefore, should be avoided unless it is absolutely necessary.Conclusion:Congenital scoliosis due to a hemivertebra at a young age could be treated by hemivertebra resection or anterior and posterior epiphysiodesis as definitive surgical treatment. The patient was eventually treated with PVCR, which achieved satisfactory correction without curve progression in a long-term follow-up.