Accessory scrotum is very uncommon. Here we report a 5-year old Caucasian boy presenting with an accessory scrotum localized to the left side of the pubic area. The penis and the primary scrotum were completely normal, containing two dependent testicles. On the right side of the body there was type B proximal femoral focal deficiency and there was also diastasis of the symphisis pubis. As observed in this case, abnormalities of two different embryological organ systems (accessory labioscrotal fold and proximal femoral focal deficiency) may occur concurrently at the same intrauterine time point as part of an overall embryologic mesenchymal disorder.