We report a unique case of bilateral proximal delta phalanges with secondary distal accessory ossification centers of the long fingers in a 16-month-old boy. The bony anomalies presented as severe ulnar deviation at the proximal interphalangeal joints bilaterally. This is a unique occurrence of congenital clinodactyly which has not been previously reported. Several family members show similar deformity, although to lesser degrees, suggesting an autosomal dominant mode of inheritance with incomplete penetrance and variable expressivity.