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Cloverleaf skull syndrome was first reported in 1960 by Holtermuller & Wiedemann as Kleeblattschadel-Syndrome. The case in the present report was that of a male infant 26 days old at the time of death. Besides a trilobed skull and abnormalities of the extremities, he had low-set ears, stenosis of both external auditory canals, a beak-like nose, microphthalmia, retentio testis and congenital dermal sinus-like anomaly at the coccygeal region. A chromosome study revealed a normal male karyotype. The right temporal bone showed some anomalies through the external and middle ears. The bony portion of the external auditory canal was narrowed. The middle ear cavity was filled by mesenchymal tissue devoid of cell components. The incus was almost attached to the lateral tympanic wall. In addition, the stapes was thickened and bent anteriorly. The sensory and neutral elements of the inner ear were normal.