Lumbo-costo-vertebral syndrome is a rare anomaly that affects the vertebral bodies, the ribs and the muscles of the abdo- minal wall, and multiple additional congenital abnormalities requiring multidisciplinary management and early surgical inter- vention to avoid complications. Case presentation: A 10-day-old male infant presents with two right lumbar tumors, one that increased in size with crying consistent with a lumbar hernia, and the other a myelomeningocele. In complementary studies, it was found that the patient had fusion of the 1st and 2nd, 3rd and 4th and 11th and 12th ribs on the right, a 13th accessory rib on the right, and a 13th and 14th accessory rib on the left in addition to multiple defects in the fusion of the lumbar and sacral vertebral bodies, and a 1cm defect in the abdominal wall with protrusion of abdominal contents. The patient was diagnosed with LCVS with an associated myelomeningocele and underwent a simple closure and repair of the abdominal defect with a bovine pericardial patch, and plasty of the myelomeningocele without complications. We present this case due to the rarity with which it is reported in the literature. Conclusions: LCVS is a rare entity that merits early surgical resolution to avoid complications, in addition to multidisciplinary management.