Congenital talonavicular coalition is reported less frequently than talocalcaneal or calcaneonavicular coalition and represent approximately 1% of all tarsal coalitions. Although reportedly transmitted as an autosomal-dominant disorder, tarsal coalition may be inherited as an autosomal-recessive trait. It has been associated with various orthopaedic anomalies, including symphalangism, clinodactyly, a great toe shorter than the second toe, clubfoot, calcaneonavicular coalition, talocalcaneal coalition, and a ball-and-socket ankle. Patients with talonavicular coalitions are usually asymptomatic and rarely undergo surgical treatment. We report the case of a 24-year-old woman with symptomatic bilateral talonavicular coalitions and previously unreported associated anomalies (nail hypoplasia and metatarsus primus elevatus) and review the relevant literature. The patient underwent surgery (calcaneocuboid joint distraction arthrodesis and a proximal plantar flexion osteotomy with a dorsal open wedge of the first metatarsal). At 1-year followup, she was pain-free with better alignment of both feet and showed radiographic consolidation of the arthrodesis. Although this condition is less likely to be clinically important than other tarsal fusions, it sometimes can be painful enough for the patient to undergo surgery.