The bladder hamartoma is an extremely rare entity. We report on its presence in a 5-year-old boy with Goldenhar syndrome. Most probably, this is the first report of a bladder hamartoma presenting with obstruction of the bladder outlet resulting in urinary retention. The obstructive lesion was resected endoscopically. This proved to be curative for the lesion, since the follow-up voiding cysto-urethrogram revealed only a negligible post-void residual volume. Although urogenital anomalies have a well-known correlation with the Goldenhar syndrome, the existence of the bladder hamartoma found in association with this syndrome, according to the best of our knowledge, has not been previously reported in the world literature. With this report being only the 11 th described case of bladder hamartoma, we highlight on the management options for this exceptional histological finding. The incidence, screening, treatment decisions and important urogenital associations of the Goldenhar syndrome are also discussed.