Myelomeningocele is a complex pathology associated with variable clinical presentations and associated anomalies. Myelomeningocele presenting as an abdominal mass is extremely rare. This case report presents such a complex case of anterior lumbosacral myelomeningocele with clinical presentation of abdominal mass along with its surgical management. A 5-month-old boy was referred to the neurosurgery department, presenting with a congenital lump on his lower back along with abdominal mass, constipation, and urogenital dysfunction. An anterior Approach exploratory laparotomy was performed by a left lower abdomen oblique incision to excise the myelomeningocele. This case report illustrates that anterior lumbosacral myelomeningocele can present, in rare cases as abdominal mass along with urogenital dysfunctions and constipation and is managed with a collaborative effort from a multidisciplinary approach including urologists, neurosurgeons, and general surgeons