Lumbo-sacral transitional vertebrae (LSTV) are the most common congenital alteration of the lumbo-sacral junction and known to significantly influence pelvic anatomy. However, the influence of LSTV on dysplasia of the hip (DDH) and the surgical treatment by periacetabular osteotomy (PAO) remains unknown. We retrospectively examined standardized standing anterior–posterior pelvic radiographs of 170 patients in 185 PAO procedures. Radiographs were examined for LSTV, lateral-central-edge-angle (LCEA), Tönnis-angle (TA), femoral-head-extrusion index (FHEI), and anterior-wall-index (AWI) and posterior-wall-index (PWI). Patients with LSTV were compared to an age- and sex-matched control group. Patient-reported outcome measurements (PROMs) were evaluated pre- and in the mean 63.0 months (range 47–81 months) postoperatively. 43 patients (25.3%) had LSTV. Patients with LSTV had significantly greater PWI (p= 0.025) compared to the matched control group. No significant differences were seen in AWI (p= 0.374), LCEA (p= 0.664), TA (p= 0.667), and FHEI (p= 0.886). Between the two groups, no significant differences were detected in pre- or postoperative PROMs. Due to the increased dorsal femoral head coverage in patients with LSTV and DDH compared to patients with sole DDH, a more pronounced ventral tilting might be performed in those patients with prominent posterior wall sign to avoid anterior undercoverage, which is a significant predictor for premature conversion to hip arthroplasty after PAO. However, anterior overcoverage or acetabular retroversion must be avoided due to the risk of femoroacetabular impingement. Patients with LSTV reported similar functional outcomes and activity after PAO as the control group. Therefore, even for patients with concomitant LSTV, which are frequent with one-fourth in our cohort, PAO is an efficient treatment option to improve clinical symptoms caused by DDH.