Objective To investigate safety and short-term effect of subtotal hepatectomy with caudate lobe as sole remnant liver in treatment of hepatolithiasis, and to analyze diagnosis and treatment process of bronchobiliary fistula after hepatectomy. Methods The clinical data of 1 patient with hepatolithiasis combined with liver atrophy-hypertrophy syndrome and caudate lobe with compensatory hypertrophy who was admitted to the Gansu Provincial People’s Hospital in August 2016 were analyzed retrospectively. The body surface area of the patient was 1.47 m2, the standard total liver volume was 1 040 cm3. According to the results of CT, the expected residual 1iver volume (caudate lobe volume) after the hepatectomy was 643 cm3, and the ratio of residual liver volume over the standard total liver was 61%. The patient received the subtotal hepatectomy with the caudate lobe as the sole remnant liver and T tube drainage. The follow-up including the postoperative complications and recurrence of calculus was performed by the regular hospital check up till September 2017. Results The subtotal hepatectomy with caudate lobe as sole remnant liver was performed successfully. The operative time and intraoperative blood loss were 280 min and 3 000 mL, respectively. The peritoneal drainage tube was removed on the 8th postoperative day with a good recovery of liver function. The postoperative pathological examination showed that there were some intrahepatic bile duct pigment stones, the bile duct wall fibrous tissue hyperplasia combined with a focal liver cells hydropic degeneration, and no canceration. The patient was discharged on the 40th postoperative day. Two months later, the T tube cholangiography showed that the inferior extremity bile duct was unobstructed and there was no residual intra- and extra-hepatic stone. The liver function was normal, then the T tube was removed. After 6 months, the patient coughed and exhausted the bilious sputum, meanwhile the sputum culture showed that there were the Escherichia coli and Streptococcus viridans, then the bronchobiliary fistula was diagnosed. After the multidisciplinary discussion, the patient received the right thoracocentesis and double abdominal drainages around liver, meanwhile, combined with the anti-inflammatory, liver protection, intravenous nutrition support, etc., the bilious sputum was obviously reduced. So far, the patient had been followed up for one year, the patient's cough, and expectoration symptoms disappeared and his condition was stable. Conclusions Caudate lobe-sparing subtotal hepatectomy in treatment of hepatolithiasis is safe and feasible, but it is possible that bronchobiliary fistula is followed after operation, individual and multidisciplinary collaboration in treatment of bronchobiliary fistula caused by extensive hepatectomy is safe and feasible.