We read with interest the article by Gleeson et al. [2004] entitled. ‘‘Molar Tooth Sign of the Midbrain–Hindbrain Junction: Occurrence in Multiple Distinct Syndromes.’’ The molar tooth sign was previously considered pathognomonic of Joubert Syndrome. The authors illustrate that this finding can be present in other syndromes as well. In the article they describe two cases with Varadi–Papp Syndrome or Orofaciodigital Syndrome type VI, where the molar tooth sign was seen. The authors state that they believe the molar tooth sign is a frequent component of this syndrome based on these two cases, and given that posterior fossa abnormalities have been previously reported in patients. Unfortunately, the authors were not able to evaluate other patients with Varadi–Papp Syndrome to determine if they also had a molar tooth sign. We would therefore like to describe our experience with a child with Varadi–Papp Syndrome. The patient was born to a G13, P10, and SA2 woman at 39-week gestation. The mother had little prenatal care but denied exposures to teratogens. One sister has a Tetralogy of Fallot secondary to a 22q11.2 deletion. A half-sister has aortic, mitral, and pulmonary valve stenosis. The parents are both of Aboriginal Canadian origin. There is no known consanguinity. The diagnosis of Varadi–Papp syndrome made was based on the presence of multiple anomalies including preand post-axial polysyndactyly, a midline cleft lip, cleft palate, small tongue, and multiple alveolar frenula. Both hands had eight digits (Fig. 1) and both feet had seven digits (Fig. 2). A Y-shaped metacarpal was noted on the left hand. Other problems included visual impairment, developmental delay, hypotonia, airway obstruction requiring tracheostomy, cryptorchidism, and a bicuspid aortic valve. He had a normal karyotype, that is, 46,XY. FISH studies showed that he did not have the 22q11.2 deletion, seen in his sister. MRI imaging was done. The superior vermis was dysplastic, with a sagital midline cleft. The superior cerebellar peduncles was thickened and mal-orientated. The anterior-posterior dimension of the pons was reduced, resulting in a deep interpeduncular cleft. These latter findings resulted in a molar tooth configuration of the upper pons (Fig. 3). The superior aspect of the fourth ventricle had a ‘‘batwing’’ configuration. This case therefore supports the conclusion of Gleeson et al. [2004] that the molar tooth sign is a frequent component of Varadi–Papp syndrome and that this syndrome should be considered as one of the Joubert Syndrome Related Disorders.