Ganglioneuromas (GNs) are rare benign tumors common in the pediatric population. Although mostly asymptomatic, some can cause symptoms, particularly neurological ones. Here, we report a case of a two-year-old male child, who presented changes in gait, an alarming sign, during a child health surveillance appointment. On physical examination, the child presented a “duck” gait pattern, axial and appendicular hypotonia, proximal weakness of the pelvic girdle, and a positive Gowers sign. The child was referred to a pediatric neurology appointment where he underwent neuraxial magnetic resonance imaging, identifying a large and expansive formation in the dorsolumbar transition suggestive of neuroblastoma, considering the age group. However, a computed tomography-guided biopsy revealed it to be a paravertebral GN. Tumor resection was performed, leaving some paravertebral tumor residue. After one year of motor rehabilitation, the child had a normal neurological examination. The child is currently five years old and is undergoing annual clinical and imaging surveillance. This case allows us to reflect on the importance of encouraging children and young people to attend recommended surveillance appointments and reminds us that the rarest situations can occur.