Perianal pyramidal protrusion is described for the first time in 1996 as a benign soft tissue protrusion, exclusively affecting the female sex, often at a young age, three types are individualized congenital, functional or associated to lichen sclerosis. This entity would be due to a constitutional weakness of the perineal wall, in particular of the median raphe, or would be secondary to a malformation of the urogenital septum during embryonic life. This would explain the exclusive involvement of the female sex and the presence of familial cases. The functional form would be secondary especially to constipation but also to diarrhea and anal fissures. Skin lesions are in most cases mistaken for condylomas, anal vestiges or even sexual abuse. It evolves often towards spontaneous regression over several weeks.

We report the observation of a female infant, 10 months old, asymptomatic. The clinical examination objectified in the anterior part of the ano-perineal area a pyramidal cutaneous protrusion of 5 mm of diameter; pink in color and with a smooth surface (Figure 1). There was no irritation, cracking, pigmentary disorders, no similar cases are reported in the family. The questioning found a notion of constipation during 1 month. We have concluded to the cause of transit disorder. The followup under treatment, skin lesion disappeared after four months.