A 7-year-old boy followed since infancy for unilateral hypoplasia of the calcaneus is reported. In the present case, which seems to be the second in the literature, the hypoplasia of the calcaneus is similar to the one previously reported, but the involvement is unilateral. In addition, the child presents the following defects: a. absence of the fifth metatarsal, b. an ectrotic fifth toe, c. generalized hypoplasia of the foot and to a minor degree of the tibia, d. abnormalities of the ear lobes, upper lip, high arched palate, and e. a mild pectus excavatum. At the age of 7 years the child is of normal intelligence. He walks with a minimal limp as he has shortening of one centimeter and slight equinus on the affected side. Nevertheless he participates in all school activities and sports.