BACKGROUND: Superior mesenteric artery syndrome causes chronic duodenal obstruction. Studies on this disease are limited; therefore, several difficulties in the diagnosis and treatment of such patients remain. AIM: This study aimed to present the experience of treating children with superior mesenteric artery syndrome. MATERIALS AND METHODS: The treatment results of 45 patients with superior mesenteric artery syndrome was retrospectively studied. The children complained of abdominal pain, nausea, occasional vomiting, belching, bloating, and constipation. The diagnosis was confirmed during a comprehensive examination, including ultrasound, esophagogastroduodenoscopy, X-ray contrast examination, computed tomography, and relaxation duodenography. Conservative therapy was performed in 38 (84,4%) children, and 21 (55,3%) children showed satisfactory results. In case of ineffectiveness of conservative measures (17 cases) or in a decompensated state (7 cases), indications for surgical treatment were provided. Furthermore, 24 (53,3%) children underwent surgery. Duodenal drainage surgeries were performed in 20 (83,3%) patients with subcompensation of duodenostasis. Of these patients, 10 (41,7%) underwent lower duodenojejunostomy with a switched-off Roux-en-Y loop (Gregory–Smirnov’s operation) and the other 10 (41,7%) underwent anterior mesenteric duodenojejunostomy (Robinson’s operation). Laparotomic access was used in 14 cases (70,0%) and laparoscopic in 6 (30,0%) cases. Owing to decompensation of duodenostasis, the duodenum was excluded from passage by economical resection of the gastric outlet with gastrojejunostomy on a short loop with additional formation of a lower duodenojejunostomy according to Roux in 4 (16,7%) cases. Laparotomic access was used in all cases. RESULTS: No intraoperative complications were noted. In the early postoperative period, two children developed anastomositis after Robinson’s operation and two patients after Gregory–Smirnov’s operation, which was treated with conservative measures. In long-term followup (up to 15 years), a satisfactory result was achieved in 87.5% of cases. CONCLUSIONS: Superior mesenteric artery syndrome is a relatively rare cause of chronic duodenal obstruction in children. When selecting patients for surgical treatment, other diseases should be excluded. Surgical correction includes various options for duodenal drainage operations that can be successfully performed using laparoscopic access. In case of decompensation of duodenostasis, it may be crucial to exclude the duodenum from the passage.