Larsen syndrome is a complex syndrome with genetic heterogeneity, and with both autosomal dominant and autosomal recessive patterns of inheritance. It is characterized by congenital dislocation of joints, flat faces and complicated by issues relating to respiratory, cardiac, musculoskeletal and central nervous systems. This report describes the anaesthetic management of two patients with Larsen syndrome. The first case is a 4-year-old patient who had cervical cord compression secondary to cervical instability and who was scheduled for anterior corpectomy with fusion of cervical vertebrae and placement of halo frame. This patient had transient loss of evoked potentials during positioning and a stormy postoperative course requiring reintubation and a prolonged stay in the intensive care unit. The second case is a 22-month-old child who was scheduled for a repeat posterior cervical spinal fusion due to failure of her initial fusion procedure. This patient had an uneventful perioperative course. Relevant anaesthetic issues in patients with Larsen syndrome are discussed.