Purpose To review recent advances in the diagnosis and treatment of congenital vascular malformations of the retina and choroid. Methods Review of the current literature on retinal haemangioblastoma, racemose haemangiomatosis, retinal cavernous haemangioblastoma, circumscribed choroidal haemangioma, and diffuse choroidal haemangioma. Conclusion The management of patients with congenital vascular lesions of the retina and choroid is advancing rapidly through recent developments in genetic testing, ocular imaging, and treatment. Most are associated with systemic disease, which may be lifethreatening. New therapeutic methods such as Verteporfin photodynamic therapy and antiangiogenic therapy have significantly improved the treatment of retinal and choroidal angiomas and vascular malformations. Ophthalmologists have a major role in detecting and diagnosing these tumours and in providing long-term care in collaboration with a specialist in the field.