A perineal fluid-filled structure was discovered in a 6-year-old intact female Irish water spaniel suffering from intermittent constipation. Diagnostic tests revealed the structure was immediately caudal to the vagina and compatible with a cyst. Surgical excision was required for resolution of clinical signs. Histology confirmed the structure was a cyst. The exact origin is unknown; however, the variety of lining epithelia, including sections with mucin production, and a well-differentiated smooth muscle layer, were most consistent with development from the lower hindgut or urogenital sinus during embryonic growth. The histologic and anatomical similarities with human retrorectal cystic hamartomas were key in establishing the diagnosis of a perineal cystic hamartoma. Following removal, constipation resolved, and the cyst did not recur.