Segmental absence of the intestinal musculature (SAIM) is a rare clinicopathological entity in childhood. The etiology of SAIM has not been fully elucidated. Cases of SAIM are mostly documented in neonate and adult population. A 10-year-old girl was admitted to our clinic with the complaint of acute abdominal pain that was not relieved on medical management. Ultrasound abdomen showed diffuse thickening of duodenum, jejunum, and transverse colon wall. There was fluid collection in the left lower abdomen. In view of unrelieved abdominal distention, acute abdominal pain, and intestinal obstruction, a midline laparotomy was performed. Fibrous bands were compressing the jejunum with a small jejunum segment that appeared ischemic and was resected. Histopathological examination of this jejunum segment was reported as SAIM—a clear cut histopathological picture of mucosa and sub-mucosa with no muscularis propria was seen.