Cloverleaf skull is a rarely seen deformity and the prognosis is poor in untreated cases. The main components of the deformity are hydrocephalus, trilobed head shape, shallow orbits, and midfacial hypoplasia. It may be seen either as an entity or in association with other syndromes, such as Apert's, Crouzon's, Pfeifer's syndromes, and .skeletal dysplasias. The presented case is in association with some skeletal anomalies, together with ambiguous genitalia and dermal sinus tract over lumbar spine. As early surgical intervention is suggested to prevent the damages of increased intracranial pressure, we operated the patient on the second day of her life. Although the vital signs of the infant returned to normal limits immediately after the operation, the patient died on the third postoperative day because of diffuse intravascular coagulopathy.