Short bowel syndrome is defined as the loss, congenital or acquired, anatomical or functional, of a large part of the small intestine that generates inadequate absorption of nutrients and the frequent need of prolonged parenteral nutrition. The etiology of short bowel is diverse and varies with age. The necrotizing enterocolitisis and the midgut volvulus are among the most frequent causes. The bacterial overgrowth is frequently observed in children with short bowel, due to the secondary dilation of the remaining small bowel and to the associated intestinal dysmotility. It is more frequent in absence of the ileocecal valve. We present a 6-year-old boy with short bowel syndrome secondary to extensive intestinal resection after a volvulous of the medium small intestine, 9 months before admission to the hospital, and who was on cyclical parenteral nutrition at home. The child developed ataxia, disarthria, dizziness and conscience compromise been admitted to de intensive care unit in deep sopor. An extensive work up including metabolic, infectious, toxicology and SNC imaging was negative except for metabolic acidosis. He was discharged on good conditions. Even though the child was on supportive therapy, the patient was readmitted few hours later with similar symptoms. D-lacto acidosis was suspected and confirmed with a value of 6.69 mmol/1 (normal range: 0,0-0,25). Literature about this uncommon complication and its mechanism is reviewed. D-lacto acidosis should be suspected in every patient with short bowel syndrome and unexplained metabolic acidosis associated with neurological symptoms.