Segmental hemangiomas may be associated with visceral hemangiomatosis, with the location of visceral lesions often correlating with the site of cutaneous involvement. Infants with large segmental hemangiomas on the face are at risk for PHACES syndrome (Posterior fossa anomalies, Haemangiomas, Arterial anomalies, Coarctation of aorta and Cardiac defects, Eye abnormalities, Sternal clefting and Supra-umbilical raphe) and should undergo a thorough ophthalmological, cardiac and neurological evaluation. Magnetic resonance imaging (MRI) with angiography of the head and neck region is usually indicated in such infants.[8910] Similar to PHACES syndrome, segmental hemangiomas in the perineal region may be associated with malformations of the urogenital or anogenital systems; these include SACRAL (spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localisation) and PELVIS (perineal haemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag) syndrome. Haemangiomas on the lumbosacral region may be a marker of occult spinal dysraphism. A spinal MRI is indicated as a screening test for all cases with lumbosacral haemangiomas.[11] Laryngeal involvement can occur if haemangiomas are present on the cervicofacial, mandibular or the ‘beard’ distribution. Affected infants should be watched for signs and symptoms of airway obstruction (stridor, hoarseness) and referred for a laryngeal examination.[12] Infants with multiple cutaneous haemangiomas (diffuse neonatal haemangiomatosis) constitute another group which is at risk for visceral involvement, and it is generally recommended to screen the patient for hepatic involvement by abdominal ultrasound.[1314] Raised levels of iodothyronine deiodinase have been demonstrated in large proliferative haemangiomas, which can lead to hypothyroidism. Till date, most of the cases of hypothyroidism have been noted in association with hepatic haemangiomatosis, though increased iodothyronine deiodinase activity has been detected in some large cutaneous lesions as well. Hence, a recommendation has been made to perform serial thyroid function tests in patients with hepatic haemangiomatosis or large cutaneous haemangiomas.[1516]