INTRODUCTION: Clinical manifestations of intestinal duplication cysts are varied, influenced by factors such as size, location, and mass effect. Most of these cysts are adjacent to the second or third portion of the native duodenum. Complete surgical excision is the standard treatment for symptomatic enteric duplication cysts. CASE PRESENTATION: We present the case of a neonate who was admitted to the hospital with abdominal distension and jaundice. Abdominal ultrasound and CT scan revealed a cystic mass of uncertain origin. Surgical exploration demonstrated a duodenal duplication cyst, which was successfully excised. Histopathological examination confirmed the diagnosis. A review of the literature highlights the approach to duodenal duplication cysts in neonates. CLINICAL DISCUSSION: While rare, duodenal duplication cysts should be considered in the differential diagnosis of abdominal masses. Comprehensive imaging studies and histopathological examination are essential for accurate diagnosis. CONCLUSION: Complete surgical excision of duodenal duplication cysts is crucial to prevent potential complications, such as malignant transformation.