Gaucher`s disease is the most common form of lysosomal storage disorder. It is caused by an inherited deficiency of beta glucocerebrosidase enzyme. It results in accumulation of beta glucocerebroside leading to end organ dysfunction. Gaucher disease is an autosomal recessive inheritance by mutations in the GBA gene on chromosome 1. It affects both sexes. The incidence is estimated to be 1:40000 in Europe. Most commonly, the disease is differentiated into a non-neuronopathic form and a neuronopathic form. Gaucher disease may present a challenge to the anesthesiologist because of abnormal coagulation and multi organ dysfunction. These factors may affect the choice of anaesthesia type. We aim to report the anaesthetic management in a 25 years old male with Gaucher’s disease posted for splenectomy.