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A full-term female neonate with ectrodactyly, polycystic kidney disease, mandibular hypoplasia, uterus unicornis, bronchogenic cyst and spina bifida is reported. The features are compared with other cases of limb and renal abnormalities reported in the literature. This case along with similar cases reported by Halal et al. (1980), Evans et al. (2000); Tobias et al. (2001); Phadke and Manisha (2006); John (2007); and Girisha et al. (2012) delineates acrorenal mandibular syndrome as a distinct entity.

Polycystic kidney disease in neonate with acrorenal mandibular syndrome