The primary cilium is a highly conserved environmental sensor and modulator of fluid movement in tubular structures. The growing recognition of mutations among its many components has led to the discovery of new disorders collectively called ciliopathies. Ciliary dysfunction disturbs a variety of signaling pathways along its basal body and axoneme that are critical for embryonic development and cell and organ homeostasis. Among the many pathways, here we discuss the emerging role of Wnt proteins in morphogenic signaling and ciliary biology during health and disease.