The cloverleaf skull anomaly represents the most manifestly extreme form of cranio-orbitofaciostenosis with hydrocephalus that has to date been associated with a uniformly poor outcome and frequent death in infancy. Assessment of the primary deformity and the approach to treatment in a consecutive series of 10 patients with the cloverleaf skull anomaly in the one unit are presented. Early predictable surgical correction of the cranio-stenosis is possible by fronto-orbital advancement and lambdoid craniectomy. Less satisfactory correction of hydrocephalus and orbitostenosis is possible with the attendant increased morbidity. Early survival and the potential for a satisfactory long-term outcome depend on the management of the faciostenosis and an airway that may be anomalous at multiple levels. The initial approach was routine tracheostomy, which provided short-term relief but with the morbidity associated with such airway maintenance in any environment other than the most sophisticated health services. More recently, upper airway surgery (uvulopalatopharyngoplasty, adenoidectomy, and soft palatal split) has ensured airway control and avoided the progression to tracheostomy. When utilized later (1 to 10 years), such surgery may alleviate the symptoms of upper airway obstruction and sleep apnea and permit delay of midfacial advancement.