A true left-sided gall bladder (LSG) is a rare finding, is mostly discovered incidentally and often presents with symptoms similar to those of a normally positioned gall bladder. The diagnosis in most cases is intraoperative. The surgical technique is frequently difficult, with an increased risk of intraoperative injuries and conversion to open surgery. In this case report, we describe a rare scenario of a young male with hereditary spherocytosis who presented with jaundice and splenomegaly. The diagnosis of LSG was made by chance during pre-operative imaging. The patient was successfully managed with a splenectomy and a cholecystectomy via the minimal access approach in the same setting.