A 53-year-old woman was referred to our hospital with a diagnosis of epigastric hernia. She had had an enlarged, bulging epigastric region and abdominal pain in the right hypochondrium and epigastrium for a year. Abdominal ultrasound showed a giant cyst and cholelithiasis. Computed tomography (CT) showed a cyst of 33 × 23 × 30 cm with content estimated at 12,000 cm3 (12 liters) and confirmed that the epigastric hernia content contained part of the cyst (A, B). The patient was subjected to resection of the cyst, cholecystectomy and Mayo repair of the epigastric hernia (). A supraumbilical median laparotomy was performed, assisted with monopolar electric scalpel. Intraoperative pathological analysis confirmed the diagnosis of a biliary cyst and gallbladder adenocarcinoma T1b. Thus, a limited 3-cm wedge resection of the gallbladder bed and lymph node dissection were performed. Skin closure was performed by primary suture with separated stitches. The patient is asymptomatic at 6 years follow-up, free of the disease and without signs of recurrence of the hernia.