Choledochal cysts (CCs) are rare congenital anomalies characterized by cystic dilatation of the intrahepatic and/or extrahepatic bile ducts. They are most commonly diagnosed in pediatric populations, with a female predominance and higher incidence in East Asian countries. The etiology is multifactorial, with the most widely accepted theory involving an anomalous pancreatobiliary junction (APBJ), which permits reflux of pancreatic enzymes into the biliary tree, leading to inflammation and ductal dilation. CCs are classified according to the Todani system into five main types (I–V), with Type I being the most prevalent. Clinical presentation varies by age and cyst type, ranging from asymptomatic cases to those with abdominal pain, jaundice, cholangitis, or pancreatitis. Diagnosis relies on imaging modalities such as ultrasound, CT, MRCP, and ERCP, which help delineate cyst morphology and guide management. Definitive treatment involves complete surgical excision of the cyst with biliary reconstruction, typically via Roux-en-Y hepaticojejunostomy. Type III cysts (choledochocele) may be amenable to endoscopic management. Long-term surveillance is essential due to the risk of malignant transformation, particularly cholangiocarcinoma, which increases with age and incomplete resection.