Introduction: situs ambiguous or heterotaxy syndrome is defined as the abnormal positioning of internal viscera relative to the normal. Diagnosis in adult is extremely rare as 90–99% of the patients have severe cardiac abnormalities and die by the age of five years. case report: A 32-year-old male was presented to hospital with sudden onset abdominal pain, abdominal distension, vomiting and absolute constipation for one day. there was no other medical problems. Examination was also unremarkable. Abdomen was distended and generally tender. Per rectal examination showed empty rectum. blood tests were unremarkable. computed tomography scan of abdomen and pelvis showed closed loop mid to distal small bowel obstruction with small bowel wall thickening. Malrotation of the bowel was noted. It also showed that stomach and spleen were on the right side of the body. Emergency laparotomy showed small bowel volvulus and spleen, stomach, duodenojejunal flexure and small bowel were in the right side of the abdomen and colon was in the left side of the abdomen. Ileocecal valve was noted in the left side of the abdomen. Adhesiolysis and derotation of the affected small bowel were performed. Appendicectomy was performed due to its