Background and Aims Long-standing hepatolithiasis with recurrent biliary inflammation may lead to liver lobe atrophy-hypertrophy syndrome. In some patients, marked atrophy of both hepatic lobes is accompanied by compensatory enlargement of the caudate lobe, making the extent of hepatectomy and accurate assessment of the future liver remnant challenging. We report a case of hepatolithiasis complicated by liver lobe atrophy-hypertrophy syndrome treated with caudate lobe-sparing subtotal hepatectomy and review the relevant literature to evaluate the indications, key technical considerations, and clinical applicability of this procedure.Methods The clinical data of a 66-year-old patient with hepatolithiasis complicated by liver lobe atrophy-hypertrophy syndrome who was admitted to the Fifth Medical Center of Chinese PLA General Hospital in March 2025 were retrospectively analyzed. Three-dimensional visualization was used preoperatively to assess hepatic morphology, caudate lobe volume, and the anatomical relationships of its vascular and biliary structures. Surgical planning was performed based on these findings and hepatic functional reserve. Caudate lobe-sparing subtotal hepatectomy combined with common bile duct exploration, stone extraction, and T-tube drainage was subsequently performed with the assistance of choledochoscopy and intraoperative ultrasonography. Relevant cases reported in the literature were also reviewed.Results Imaging revealed marked atrophy of the left and right hepatic lobes, compensatory enlargement of the caudate lobe, and multiple intrahepatic biliary stones with intrahepatic bile duct dilatation. The caudate lobe had preserved blood supply, venous drainage, and biliary drainage. Three-dimensional reconstruction showed a caudate lobe volume of 948 mL, corresponding to approximately 84% of the standard liver volume after resection. Caudate lobe-sparing subtotal hepatectomy combined with common bile duct exploration, stone extraction, and T-tube drainage was successfully performed. The operative time was 420 min, with estimated blood loss of approximately 800 mL and no blood transfusion. Liver function recovered well postoperatively, with no severe complications, and the patient was discharged on postoperative day 12. Histopathological examination unexpectedly revealed intrahepatic cholangiocarcinoma, large-duct type, with negative resection margins. At 2 months postoperatively, no definite residual stones or tumor recurrence was detected. Choledochoscopy at 4 months showed no residual biliary stones, and the T-tube was removed. During the 8-month follow-up, the patient remained in good general condition, with no definite evidence of recurrent stones or tumor on imaging.Conclusion In carefully selected patients with hepatolithiasis complicated by liver lobe atrophy-hypertrophy syndrome, caudate lobe-sparing subtotal hepatectomy is feasible after comprehensive assessment of hepatic functional reserve, future liver remnant volume, and the vascular, venous, and biliary drainage of the caudate lobe. Three-dimensional visualization, choledochoscopy, and intraoperative ultrasonography may facilitate precise preoperative planning and intraoperative identification and preservation of critical structures. However, this technically demanding procedure requires strict patient selection, and long-term complications such as intrahepatic cholangiocarcinoma should be carefully considered.