Larsen syndrome is a rare congenital anomaly; the underlying defect is thought to be a collagen malformation resulting in musculoskeletal deformities, cardiac defects and airway abnormalities such as severe tracheomalacia. In this case report we aimed to point out the characteristics of anaesthetic management at Larsen syndrome and discuss the anaesthetic technic that was applied. The patient was 3 years old boy with 10 kg body weight and 54 cm height, underwent surgery for two times because of right and left pes equinovarus deformities within three months intervals. Physical examination revealed characteristic facial abnormalities, thoracic and lumbar scoliosis and normal cervical spine mobility. The heart auscultation showed 2/6 systolic ejection and 1/6 diastolic murmur and loud S2. An echocardiography revealed atrial septal defect (ASD) and aortic root dilatation. Anaesthesia was induced with 50 % N2O/O2 in fresh gas flow and sevoflurane gradually increased via mask ventilation. Caudal block was performed with 10 mL of 0.25 % bupivacaine. Anaesthesia was maintained with IV propofol infusion as additional to caudal block. As a result in these cases an attentive preoperative anaesthetic assessment should be performed because of the orthopaedic and the other organ abnormalities. Aside from the avoidance of succinylcholine, there does not seem to be an inherent advantage to any particular general anaesthetic technique. We propose application of caudal anaesthesia and İV sedation together rather than general anaesthesia for the lower abdominal and extremity operations regarding its advantages.