Priapism is a pathological condition of a penile erection that persists beyond or is unrelated to sexual stimulation. Priapism is broadly classified into two types: (i) ischaemic priapism (veno-occlusive) (low-flow), (ii) nonischaemic priapism (arterial) (high-flow). We report the case of a newborn presenting with priapism on the first day of life and also review published data on the management and follow-up of this condition.