Neurofibromatosis type I and Becker nevus syndrome are very rare disorders as an entity, but co-occurrence of Neurofibromatosis type I and Becker nevus syndrome in a pregnant woman is exceptional and a real monitoring and management challenge. We report a case of Neurofibromatosis type I and Becker nevus syndrome with severe scoliosis in a 24 year primiparous gravida, 25 weeks of gestation. This case was marked by the severe spine deformity, in “C” shape associated with café-au-lait skin spots and two larger skin lesions, one of them hypertrichotic and with multiple papillomas. Also, the patient presented a restrictive respiratory dysfunction, worsened during pregnancy and decompensated on labor onset. This case is particular not only by its rarity but especially by the amplitude of the symptomatology. In our opinion, the overlap of Neurofibromatosis type I and Becker nevus syndrome, which individually involve changes in the spine, had an augmentative role in the severe deformity that was reached in this case. In the era of modern medicine, we faced a feminine, 2022 version of “The Hunch-back of Notre Dame” due to the absence of an early diagnosis.