Gastroschisis, a congenital periumbilical abdominal wall defect/malformation, allows intrauterine herniation of intestines at the right side of the umbilical cord. Its pathogenesis is still blurred as no tenable explanations or specific causes are found that can elucidate its increasing incidence presently in place among teenager and very young pregnant women. Left-gastroschisis is a rare variant of the commoner right-sided gastroschisis. It is sporadic in its prevalence and has as well an etiology unknown. When left-sided gastroschisis is present, one should be aware that an increased number of associated extra-intestinal abnormalities are likely. Our case has HIV exposure as a potential factor that could have had a role in the ontogenesis of the defect.