[eng] Internal carotid artery agenesis (ICA) and growth hormone deficiency in childhood are both very rare entities. We present the case of a 4-year-old boy with a polymalformation syndrome including an ICA in its less commonly reported variant, which was incidentally diagnosed in a magnetic resonance imaging study performed due to growth hormone deficiency and probable gonadotrophin deficiency. The radiological diagnosis of this infrequent entity is discussed, as well as its association with hormone deficiency due to a possible embryological disorder of the pituitary gland.