Purpose A type 2 recombinant human bone morphogenetic protein (rhBMP2) and Masquelet's procedure were used in three children presenting with congenital pseudarthrosis of the tibia (CPT). Recent studies on CPT suggested the presence in situ of pathologic tissues promoting pseudarthrosis. The authors hypothesized that large segmental resection of pseudarthrosis could improve prognosis of the CPT. Masquelet's procedure and rhBMP2 have been advocated for the treatment of long bone defect. Method The authors report three cases of CPT in children treated with Masquelet's procedure and application of rhBMP2. They analyzed all published cases of CPT similarly treated. Results In the present study, Masquelet's procedure did not improve the results in the treatment of CPT, but segmental bone reconstruction was possible. Bone healing was obtained in three out of the five applications of rhBMP2. In one case, the patient's parents asked for leg amputation. Analysis of the 33 published cases with the application of BMP in CPT points to a 62 % healing rate in this pathology. Conclusion The authors confirmed that segmental bone reconstruction is possible in CPT using Masquelet's procedure. In the literature, the success rate of the application of rhBMP in CPT appears to be lower than the healing rate usually reported without BMP. Nevertheless, the strict selection of patients, limited number of cases, and their heterogeneity make interpreting the results difficult. However, the theoretical risk which the children are exposed to during the use of BMP makes rigorous selection of the indications necessary. Finally, the interest of rhBMP2 application in Masquelet's procedure remained to be proven.