A 28-year old woman presented with Léri's disease (melorheostosis) and the rare combination of complex vascular malformations and lymphatic anomalies. Multifocal melorheostosis was segmental and unilateral, located in the left axial and peripheral skeleton, fifth thoracic vertebral body, fifth rib. left upper limb and lumbosacral spine (third lumbar body to first sacral segment). Sacral involvement was associated with spinal canal stenosis. Additionally the patient had multiple nevi and had suffered from left hemiplegia since birth. Lymphangiectasia of the mesentery and thorax led to chylothorax resistant to therapy for which the patient underwent a pleuropericardiectomy. Death ensued due to respiratory failure.