The association of Wilms tumor (WT) and vertebral disorders, such as hemivertebrae or fusion anomalies, have been described in literature. Here, we report a rare association of butterfly T3, T8 vertebrae and a T2, T7 hemivertebrae in a patient with WT, determined during initial examinations of tumoral extension. In patients with WT, investigation of vertebral malformations should be a part of diagnostic work-up.