ABSTRACT : A 26 year old female presented with recurrent episode of jaundice, anaemia and pain abdomen. She was diagnosed with hereditary spherocytosis and cholelithiasis. She had underwent ERCP with stent under general anaesthesia 2 months prior to admission at our institute

METHODS : On pre anaesthetic evaluation we found thrombocytosis with normal coagulation profile.USG showed distended gallbladder, splenomegaly 17cm and left ovarian cyst 32*27cm. She was preoxygenated with 100% oxygen for 3 min, intubated with porter ETT no 7.5 and anaesthetized. Skeletal muscle relaxants and intermittent seroflurane was given. Reversal of anaesthesia was done and she was extubated

RESULTS : we have successfully managed a patient with cholelithiasis with known case of hereditary spherocytosis posted for cholecystectomy and spleenectomy.Anaesthetic goals include avoidance of hypoxia, acidosis, and hypothermia.

CONCLUSIONS : This case is presented for rarity of presentation and anaesthetic management. Peri operative management of HS largely depends on the severity of anaemia and the degree of haemolysis. Anaesthetic goals include avoidance of hypoxia, acidosis, and hypothermia.