PurposeCaruncle dysgeneses are extremely rare and must be differentiated from caruncular and conjunctival tumors. Very few case reports with histopathological descriptions exist. In this case series, four patients with five caruncle dysgeneses, two with histopathological findings, are characterised.ObservationsPatient 1, a 26-year-old woman, presented with a conjunctival change at the left lower eyelid she had first noticed seven months earlier. She reported foreign body sensation and itching. On her left eye was a subtarsal conjunctival tumour measuring approximately 4 × 4 mm with whitish sebaceous gland-like inclusions located almost in the fornix morphologically resembling the nearby caruncle. The patient was asymptomatic after excision. Histopathological examination of the excised tissue showed non-keratinizing squamous epithelium with goblet cells. Subepithelially, there was lymphoplasmacytic cellular infiltration with intervening epidermal cysts adjacent to sebaceous glands and underlying adipose tissue, but no hair follicles or sweat/lacrimal glands. The epidermal cysts contained scattered hairs. A diagnosis of supernumerary caruncle was made.Patient 2, a 56-year-old woman, was referred for evaluation of a caruncle tumour that was reported to be present since childhood. Clinically the 5 × 5 mm measuring tumour appeared yellowish and less reflective compared to the normal caruncle tissue. Histopathologically, non-keratinizing squamous epithelium with goblet cells was found. In the area of more exposed tumour tissue, there were significantly fewer goblet cells and incipient keratinization of the superficial epithelial layers. Subepithelially, sebaceous glands and adipocytes were present. Hair follicles or sweat/lacrimal glands were not evident. A diagnosis of megacaruncle was made.Patient 3, a 58-year-old woman with Goldenhar syndrome, was clinically diagnosed with a supernumerary caruncle on the right eye as an incidental finding.Patient 4, a 24-year old man, clinically presented with a megacaruncle on the right eye and a supernumerary caruncle on the left.ConclusionsCaruncle dysgeneses are often asymptomatic and have to be differentiated from other caruncular and conjunctival tumors. If they are present, attention should be paid to signs of an oculo-auriculo-vertebral spectrum as Goldenhar syndrome. In case of unclear findings or complaints, excision with subsequent histopathological examination is required.