Larsen syndrome is a rare inherited disease associated with dislocations of multiple joints, typical syndromic facies, and multiple spine abnormalities. They often required multiple corrective orthopedic surgeries to regain their functional ability, thus needing repeated anesthesia. Apart from skeletal deformities, they have predicted difficult airway and need extreme care during intubation and positing of the patient. Abnormal posturing due to spinal deformity and poor pulmonary reserve due to kyphoscoliosis creates an extremely challenging situation for the anesthetist to manage the case during the perioperative period. Here we are describing the perioperative anesthetic management of a patient with Larsen syndrome.