A 57-year-old woman, who had oesophageal leiomyomatosis diagnosed 2 years ago, was referred for the management of a perianal lesion thought to be full-thickness rectal prolapse. The skin overlying the surface of this lesion led us to consider a circumferential plexus of external haemorrhoids as a differential diagnosis (Fig. 1). However, partial excision of the lesion revealed no haemorrhoidal tissue and demonstrated a rubbery and encapsulated lesion (Figs. 2, 3, 4, 5). Histological examination of the excised specimen established the unusual diagnosis of anorectal leiomyomatosis (Figs. 6, 7).