This is an 18-year (1985–2001) retrospective review of 85 patients with true hermaphroditism, with the aim of facilitating early recognition of this condition. Presentation of neonates and infants 6 months or younger, constituting 54% of this cohort, were different from the older children. The presentation, clinical features and investigative results of all patients diagnosed with true hermaphroditism at a single South African paediatric surgical unit were reviewed. This paper highlights the previously reported high incidence (51%) of this condition, as well as some of the unusual features of true hermaphroditism in this region. Diagnosis of true hermaphroditism requires a high index of suspicion for subtle anomalies of the genitalia. Although there were no pathognomonic clinical features, the true hermaphrodite presents as a patient of either gender with a congenital anomaly of the genitalia. The child is likely to have a normal male phallus, bifid labio-scrotal folds, a perineal hypospadias and in 53% of patients there was a palpable gonad. The method of investigation, together with the results and some of the management dilemmas associated with true hermaphroditism in a Third World population are presented.