Congenital atrophic plaques are an uncommon finding, observed in an asymmetric pattern with linear distribution in Goltz syndrome (focal dermal hypoplasia). We report on a patient proposed to have Goltz syndrome whose skin biopsy suggested a more appropriate diagnosis of porokeratotic eccrine ostial and dermal duct nevus syndrome (PEODDN). Our proband was a 5 month old female with congenital atrophic plaques distributed over her neck, cheek, chest, legs and perianal area in a linear fashion. She was nondysmorphic and had normal development. The diagnosis of Goltz syndrome was suggested. Subsequent evaluations for ocular defects and renal anomalies were normal, as was a routine blood karyotype. She continued to have eruptions of new lesions, leading to a skin biopsy. Histology demonstrated deep epidermal invaginations filled with a parakeratotic columns, the hallmark histologic features of PEODDN. PEODDN is a rare dermatologic condition with punctuate pits and keratotic erythematous papules on the palms and soles. More widespread truncal involvement, as was present in our patient, has been reported. There are no congenital anomalies associated with the skin lesions, in contrast to Goltz syndrome, which can have involvement of the eyes, kidneys, and extremities. PEODDN is sporadic, which also distinguishes it from Goltz syndrome, an X-linked dominant condition. Although clinical features and dermatologic findings are similar in Goltz syndrome and PEODDN, differences in systemic involvement and inheritance patterns are distinct. We suggest that the diagnosis of PEODDN be considered in all patients in whom a diagnosis of Goltz syndrome is entertained.